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Richard Bridgen
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Investigation and management of thrombocytopenia in pregnancy.
Resources searched
NHS Evidence; TRIP Database; Cochrane Library; AMED; BNI; CINAHL; EMBASE; MEDLINE;
Google Scholar
Database search terms: thrombucytopaenia; exp THROMBOCYTOPENIA;
thrombocytopen*; thrombopen*; “low platelet count”; pregnan*; exp PREGNANCY; labor;
labour; childbirth; “child birth”; gestation; gravid*; trimester*; pre-eclampsia; eclampsia;
exp ECLAMPSIA; investigation*; dianos*; management; MANAGEMENT; treatment*.
therap*
Google search string: (thrombocytopenia OR thrombocytopenic OR thrombopenia OR
FNAIT OR TTP OR ITP) (pregnant OR pregnancy OR foetus OR fetus OR fetal OR foetal)
Summary
There is a great deal of research into thrombocytopenia, its subtypes and pregnancy, even
within the last three years. I have included foetal and FNAIT research as this relates to
pregnancy but have omitted those papers dealing with purely neonatal
thrombocytopenia. I hope you find it useful.
1
Guidelines
British Committee for Standards in Haematology
Guideline on the investigation and management of adults and children presenting with
thrombocytosis 2010
ET is the commonest MPN in women of childbearing age and a significant number of
pregnancies have been described in the literature, but these data do not enable confident
management guidelines to be drawn up. A summary and suggested algorithm for
pregnancy management in ET
European Society of Cardiology
Guidelines on the management of cardiovascular diseases during pregnancy 2011
Heparin-induced thrombocytopenia is markedly lower with LMWH than with UFH as is
heparin-induced osteoporosis (0.04%).24
Finnish Medical Society Duodecim
Thrombocytopenia 2007
New South Wales Health
Maternity - management of hypertensive disorders of pregnancy 2011
1. If features of preeclampsia are present, additional investigations should include:

If there is thrombocytopenia or a falling haemoglobin, investigations for
disseminated intravascular coagulation (coagulation studies, blood film, LDH,
fibrinogen).
2. Thrombocytopenia is the commonest hematologic abnormality seen in preeclampsia;
the lower limit of the normal platelet count in pregnancy is approximately 140x109/L
but the risk of spontaneous bleeding is not significantly increased until the count falls
below 50 x 109/L. Even so, there are concerns with central neuraxial anaesthetic and
analgesic techniques at higher levels (50-75 x 109 /L), and surgical bleeding may be
increased even with moderate thrombocytopenia…
NICE
CG107 Hypertension in pregnancy 2011
1. Chlorothiazide may carry the risk of congenital abnormality, neonatal
thrombocytopenia, hypoglycaemia and hypovolaemia.
2. Neonatal thrombocytopenia and bleeding secondary to hydralazine ingestion
throughout the 3rd trimester have been reported in 3 infants. This however may have
been due to maternal hypertension.
CG92 Venous thromboembolism - reducing the risk 2011
Fondaparinux + GCS was the most cost-effective strategy when heparin Induced
thrombocytopenia was considered.
Royal College of Obstetricians and Gynaecologists
Thrombosis and Embolism during Pregnancy and the Puerperium, Reducing the Risk 2009
1. Danaparoid is a heparinoid that is mostly used in patients intolerant of heparin, either
because of heparininduced thrombocytopenia or a skin allergy to heparins…The
experience in the use of this agent in a total of 51 pregnancies was reviewed… There
were four maternal bleeding events, two of which were fatal owing to placental
problems (praevia and abruption)…There were no adverse fetal outcomes attributed
to danaparoid.
2
2. Lepirudin. Its use is best avoided in pregnancy unless there is no acceptable
alternative.
Thromboembolic disease in pregnancy and the puerperium: acute management 2007
Pregnant women who develop heparin-induced thrombocytopenia or have heparin allergy
and require continuing anticoagulant therapy should be managed with the heparinoid,
danaparoid sodium or fondaparinux, under specialist advice.
SIGN
Prevention and management of venous thromboembolism 2010
2.9.1 heparin induced thrombocytopenia
1. Monitoring patients for the development of HIT should be by performing serial
platelet counts. Patients who have previously received UFH or LMWH within 100 days
or in whom the history of recent exposure to heparins is not clear should have a
platelet count performed within 24 hours of receiving the first dose of treatment.
2. All other patients for whom monitoring is indicated should have platelet counts
performed every two to three days from day four to day14 of exposure.
3. In patients with HIT, alternative anticoagulation should be provided irrespective of
whether or not there is evidence of a new thrombotic event unless the risk of
haemorrhage is deemed excessive.419,420 Two drugs, lepirudin421,422 and
danaparoid,419 are currently licensed in the UK for immediate management of this
condition.
Society of Obstetricians and Gynaecologists of Canada
Diagnosis, evaluation and management of the hypertensive disorders of pregnancy 2008
The third stage of labour should be actively managed with oxytocin 5 units IV or 10 units
IM, particularly in the presence of thrombocytopenia or coagulopathy. (I-A)
Society of Obstetric Medicine od Australia and New Zealand
Guidelines for the management of hypertensive disorders of pregnancy 2008
1. When relatively contraindicated (e.g. severe thrombocytopenia, coagulopathy or
sepsis), fentanyl or remifentanil patient-controlled intravenous analgesia is preferred.
2. … there are concerns with central neuraxial anaesthetic and analgesic techniques at
higher levels (50-75 x 109 /L), and surgical bleeding may be increased even with
moderate thrombocytopenia…
Evidence-based reviews
American College of Obstetricians and Gynecologists
Medical management of ectopic pregnancy 2011
Absolute Contraindications to Methotrexate Therapy - Preexisting blood dyscrasias, such
as bone marrow hypoplasia, leukopenia, thrombocytopenia, or significant anemia
BJOG: An International Journal of Obstetrics & Gynaecology
Screening in pregnancy for fetal or alloimmune thrombocytopenia: systematic review
2010
Screening for HPA-1a alloimmunisation detects about two cases in 1000 pregnancies. The
calculated risk for perinatal ICH of 10% in pregnancies with severe FNAIT is an
underestimation because studies without interventions were lacking. Screening of all
pregnancies together with effective antenatal treatment such as intravenous
3
immunoglobulin may reduce the mortality and morbidity associated with FNAIT.
Clinical Knowledge Summaries
Antiplatelet treatment 2009
1. What are the adverse effects of dipyridamole? - Thrombocytopenia (isolated case
reports).
2. Do not prescribe aspirin - People with haemophilia or another haemorrhagic disorder
(including thrombocytopenia).
Cochrane Database of Systematic Reviews
Antenatal interventions for fetomaternal alloimmune thrombocytopenia 2011
The optimal management of fetomaternal alloimmune thrombocytopenia remains
unclear. Lack of complete data sets for two trials and differences in interventions
precluded the pooling of data from these trials which may have enabled a more
developed analysis of the trial findings. Further trials would be required to determine
optimal treatment (the specific medication and its dose and schedule). Such studies
should include long-term follow up of all children and mothers.
Medical treatments for idiopathic thrombocytopenic purpura during pregnancy 2009
Current evidence indicates that compared to no medication, betamethasone did not
reduce the risk of neonatal thrombocytopenia and neonatal bleeding in ITP during
pregnancy. There is insufficient evidence to support the use of betamethasone for
treating ITP. This Cohrane review does not provide evidence about other medical
treatments for ITP during pregnancy. This systematic review also identifies the need for
well-designed, adequately powered randomised clinical trials for this medical condition
during pregnancy. Unless randomised clinical trials provide evidence of a treatment effect
and the trade off between potential benefits and harms are established, policy-makers,
clinicians, and academics should not use betamethasone for ITP in pregnant women. Any
future trials on medical treatments for treating ITP during pregnancy should test a variety
of important maternal, neonatal or both outcome measures, including maternal death,
perinatal mortality, postpartum haemorrhage and neonatal intracranial haemorrhage.
Health Technology Assessment Database
The clinical effectiveness and cost-effectiveness of enzyme replacement therapy for
Gaucher’s 2007
1. Anaemia, thrombocytopenia and leucopenia are common presenting features of type
I Gaucher’s disease.
2. The results of this RCT support the conclusion that ERT delivers beneficial effects for
anaemia, thrombocytopenia and hepatomegaly, but that inducing changes in bone
marrow may in fact exacerbate the development of osteopenia at certain sites in
splenectomised adults.
NHS Economic Evaluation Database
Cost-effectiveness of antenatal screening for neonatal alloimmune thrombocytopenia
2007
The antenatal screening programme for neonatal alloimmune thrombocytopenia (NAIT)
was found to be cost-effective in comparison with no screening. However, the net costs
and benefits of the three strategies were fairly similar, and the study did not give clear
guidance as to which one would be preferable.
Prospective epidemiologic study of the outcome and cost-effectiveness of antenatal
4
screening to detect neonatal alloimmune thrombocytopenia due to anti-HPA-1a 2006
The effectiveness analysis showed that antenatal screening was more effective in
detecting NAIT cases than no screening.
NHS Evidence
Antenatal interventions for fetomaternal alloimmune thrombocytopenia 2011
Evidence shows that the harms of treating fetomaternal alloimmune thrombocytopenia
with dexamethasone alone, or in combination with intravenous immunoglobulin may
outweigh the benefits.
Reducing or stopping antenatal administration of dexamethasone in fetomaternal
alloimmune thrombocytopenia is likely to improve the quality of patient care and result
in productivity savings by avoiding an unproven intervention with an unknown safety
profile.
UK Clinical Trials Gateway
Interventional Study in Adult Subjects With Immune Thrombocytopenia Purpura Receiving
Romiplostim 2010
The purpose of this study is to describe the number of months with a subject platelet
response over a 12 month treatment period and to describe ITP Remission Rates in Adult
Subjects with Immune Thrombocytopenia Purpura Receiving Romiplostim
UK National Screening Committee
Thrombocytopenia 2006
Screening for this condition should not be offered. This policy was reviewed in Jul 2006
but no significant changes were made. It is due to be considered again in 2010/11, or
earlier if significant new evidence emerges.
Published research
CINAHL results
66. Thrombocytopenia in pregnancy: making the differential diagnosis.
Author(s): Berkley EMF, Kilpatrick SJ
Citation: Contemporary OB/GYN, 01 January 2009, vol./is. 54/1(36-41), 00903159
Publication Date: 01 January 2009
Abstract: When the platelet count drops, separating benign from life-threatening causes
can prove quite challenging. Two experts explain the differences between gestational
thrombocytopenia, heparin-induced thrombocytopenia, disseminated intravascular
coagulopathy, idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic
purpura, and other disorders.
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
67. Epidemiology, pathophysiology, and initial management of chronic immune
thrombocytopenic purpura.
5
Author(s): Pruemer J
Citation: American Journal of Health-System Pharmacy, 03 January 2009, vol./is. 66/2(0-),
10792082
Publication Date: 03 January 2009
Abstract: Purpose. The incidence and epidemiology, the pathogenesis, the clinical
symptoms and diagnosis and the first-line therapies for the management of chronic
immune thrombocytopenic purpura (ITP) are discussed. In addition, the recommendations
of two expert panels for the management of ITP are summarized.Summary. The diagnosis
and management of chronic ITP are a challenge to the clinician caring for patients with
this disease. Because the pathophysiology of ITP is not completely understood, a variety
of medical interventions have been utilized in the management of ITP. National guidelines
have established that oral corticosteroids are considered to be first-line therapy for
chronic ITP. In addition, the use of intravenous immune globulin has demonstrated
efficacy in the treatment of the disease. Intravenous methylprednisolone, anti-D
immunoglobulin, and splenectomy have been utilized in recurrent or refractory cases. The
use of other immunosuppressant medications and newer thrombopoietin stimulating
agents may offer additional treatment options, as presented in the subsequent
article.Conclusion. The initial management of chronic ITP should consist of the use of oral
corticosteroids according to national guidelines. In the absence of a response to this firstline therapy, intravenous gamma globulin, intravenous methylprednisolone, anti-D
immunoglobulin, or splenectomy may be considered. These treatments may also be
utilized to manage recurrent cases of ITP, prior to consideration of second-line therapies.
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
Available in fulltext at EBSCO Host
68. Maternal thrombocytopenia-absent radius syndrome complicated by severe preeclampsia.
Author(s): Wax JR, Crabtree C, Blackstone J, Pinette MG, Cartin A
Citation: Journal of Maternal-Fetal & Neonatal Medicine, 01 February 2009, vol./is.
22/2(175-177), 14767058
Publication Date: 01 February 2009
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
69. The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic
uremic syndrome in the obstetric triage and emergency department: lessons from 4
tertiary hospitals.
Author(s): Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, How H, Sibai B
Citation: American Journal of Obstetrics & Gynecology, 01 April 2009, vol./is. 200/4(0-),
00029378
Publication Date: 01 April 2009
Abstract: OBJECTIVE: We report a series of occurrences of thrombotic thrombocytopenic
purpura (TTP)/hemolytic uremic syndrome (HUS) in pregnancy that emphasizes early
6
diagnosis. STUDY DESIGN: Fourteen pregnancies with TTP (n = 12) or HUS (n = 2) were
studied. Analysis focused on clinical and laboratory findings on examination, initial
diagnosis, and treatment. RESULTS: There were 14 pregnancies in 12 patients; 2 cases of
TTP were diagnosed as recurrent. Five women were admitted to the emergency
department (ED), and 7 patients were admitted to an obstetrics triage. Patients who were
evaluated by an obstetrician were treated initially for hemolysis, elevated liver enzymes
and low platelets syndrome/preeclampsia, whereas patients who were seen in the ED had
a diagnosis that is commonplace in the ED (panic attack, domestic violence,
gastroenteritis). Latency from the onset of symptoms to diagnosis ranged from 1-7 days.
Plasmapheresis treatments in early gestation resulted in favorable maternal-neonatal
outcome. Maternal and perinatal mortality rates were 25% each. CONCLUSION: TTP/HUS
is a challenging diagnosis in obstetric triage and ED areas. We propose a management
scheme that suggests how to triage patients for early diagnosis in pregnancy.
Source: CINAHL
70. Regional anesthesia in the parturient with idiopathic thrombocytopenia purpura.
Author(s): Shaw MB
Citation: International Student Journal of Nurse Anesthesia, 01 August 2009, vol./is.
8/2(46-49),
Publication Date: 01 August 2009
Source: CINAHL
71. Prenatal treatment of fetomaternal thrombocytopenia.
Author(s): Vatopoulou T, Sorinola O
Citation: British Journal of Hospital Medicine (17508460), 01 November 2009, vol./is.
70/11(660-661), 17508460
Publication Date: 01 November 2009
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
Available in print at Lincoln County Hospital Professional Library
72. Is it time to implement HPA-1 screening in pregnancy?
Author(s): Husebekk A, Killie MK, Kjeldsen-Kragh J, Skogen B
Citation: Current Opinion in Hematology, 01 November 2009, vol./is. 16/6(497-502),
10656251
Publication Date: 01 November 2009
Abstract: PURPOSE OF REVIEW: The purpose of the review is to argue for and against
introduction of HPA-1 typing of all pregnant women to reduce morbidity and mortality
caused by foetal/neonatal alloimmune thrombocytopenia (FNAIT). RECENT FINDING:
Several groups have done HPA-1 typing in cohorts of pregnant women. Results from a
Norwegian study (>100,000 pregnancies) indicate that screening combined with simple
intervention decreases morbidity and mortality due to FNAIT and is cost effective in
Norway. Results from this study and several other studies show that there is correlation
between the level of anti-HPA-1a antibodies in the mother and the severity of
thrombocytopenia in the newborn. An important finding is that about 75% of women with
antibodies are immunized in connection with delivery. Only 25% of the women are
7
immunized during pregnancy. SUMMARY: Screening for FNAIT does not fully meet the
criteria presented by the WHO. Nevertheless, the results of the Norwegian study strongly
indicate that morbidity and mortality related to FNAIT can be reduced. If the recent
attempts to make a vaccine aimed at prevention of immunization and/or tolerizing
peptides or neutralizing antibodies for already immunized women are shown to be
successful, screening must be implemented.
Source: CINAHL
73. Perinatal outcomes and complications of pregnancy in women with immune
thrombocytopenic purpura.
Author(s): Belkin A, Levy A, Sheiner E
Citation: Journal of Maternal-Fetal & Neonatal Medicine, 01 November 2009, vol./is.
22/11(1081-1085), 14767058
Publication Date: 01 November 2009
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
74. Medical treatments for idiopathic thrombocytopenic purpura during pregnancy.
Author(s): Martí-Carvajal AJ, Peña-Martí GE, Comunián-Carrasco G
Citation: Cochrane Database of Systematic Reviews, 01 December 2009, vol./is. /4(0-),
1469493X
Publication Date: 01 December 2009
Abstract: Background:
Source: CINAHL
Full Text:
Available in fulltext at Wiley
75. Immune thrombocytopenia in pregnancy.
Author(s): Stavrou E, McCrae KR
Citation: Hematology/Oncology Clinics of North America, 01 December 2009, vol./is.
23/6(1299-1316), 08898588
Publication Date: 01 December 2009
Abstract: Management of immune thrombocytopenia in pregnancy can be a complex and
challenging task and may be complicated by fetal-neonatal thrombocytopenia. Although
fetal intracranial hemorrhage is a rare complication of immune thrombocytopenia in
pregnancy, invasive studies designed to determine the fetal platelet count before delivery
are associated with greater risk than that of fetal intracranial hemorrhage and are
discouraged. Moreover, the risk of neonatal bleeding complications does not correlate
with the mode of delivery, and cesarean section should be reserved only for obstetric
indications.
Source: CINAHL
76. Neonatal outcomes of pregnancy complicated by idiopathic thrombocytopenic
purpura.
8
Author(s): Ozkan H, Cetinkaya M, Koksal N, Ali R, Gunes AM, Baytan B, Ozkalemkas F,
Ozkocaman V, Ozcelik T, Gunay U, Tunali A, Kimya Y, Cengiz C
Citation: Journal of Perinatology, 01 January 2010, vol./is. 30/1(38-44), 07438346
Publication Date: 01 January 2010
Abstract: Objective:The aim of this study was to determine the factors associated with the
prognosis of newborns born to mothers with idiopathic thrombocytopenic purpura (ITP),
and to compare the infants with/without thrombocytopenia in terms of maternal and
neonatal characteristics.Study Design:We reviewed the charts of 29 parturients with ITP
and their newborns who were born between January 1998 and December 2008.Result:A
total of 16 (55%) gravidas had been diagnosed with ITP before pregnancy and 13 (45%)
were diagnosed during pregnancy. Thrombocytopenia was observed in 21 gravidas. In
total, 17 (58%) gravidas received treatment to increase the platelet count. The majority of
deliveries (72.5%) were vaginal. The infant platelet counts at birth ranged from 20 to 336 x
109 per liter. None of the neonates had complications attributable to the mode of
delivery. Normal platelet counts were determined in 15 newborns, whereas 14 infants had
thrombocytopenia at birth. Three (10.3%) neonates had mild, four neonates (13.7%) had
moderate and seven neonates (24.1%) had severe thrombocytopenia. The age of the
mothers having infants with thrombocytopenia was significantly higher (30-5.3 vs 25.3-3.8
years), most of the infants (10/14 (71%)) were males (P<0.05).Conclusion:Pregnancy
complicated with ITP generally has a good outcome. Although ITP in pregnancy carries a
low risk, careful observation is required for the newborn of gravidas with ITP even when
the infant has no bleeding complications at delivery, and infants may require treatment
for thrombocytopenia.
Source: CINAHL
77. HELLP syndrome -- a case study.
Author(s): Barnett R, Kendrick B
Citation: New Zealand Journal of Medical Laboratory Science, 01 April 2010, vol./is.
64/1(14-17), 11710195
Publication Date: 01 April 2010
Abstract: Background: HELLP syndrome is a life-threatening obstetric complication
considered by many to be a severe form of preeclampsia involving haemolysis,
thrombocytopenia and liver dysfunction. Both HELLP and pre-eclampsia occur during the
later stages of pregnancy, and sometimes after childbirth. HELLP syndrome is a clinically
progressive condition. Early diagnosis is critical to prevent liver distension, rupture and
haemorrhage and the onset of Disseminated Intravascular Coagulation. If the condition
presents antenatally, morbidity and mortality can affect both mother and baby. Case
study: We report a case study of HELLP syndrome in a 40 year old post-partum woman,
who had evidence of pre-eclampsia in the weeks leading up to delivery. Within 24 hours
of delivery the patient's blood pressure was rising significantly, she experienced upper
epigastric pain and vomiting, her platelet count was dropping rapidly and blood tests
revealed that her liver enzymes were becoming increasingly abnormal. All of these
symptoms led to the rapid diagnosis of HELLP syndrome. Conclusions: This case is of
interest because if the diagnosis had been delayed, there could have been a significant
risk of liver rupture, DIC or haemorrhage, and patient survival could have been
compromised.
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
9
78. The use of angiogenic biomarkers to differentiate non-HELLP related
thrombocytopenia from HELLP syndrome.
Author(s): Young B, Levine RJ, Salahuddin S, Qian C, Lim KH, Karumanchi SA, Rana S
Citation: Journal of Maternal-Fetal & Neonatal Medicine, 01 May 2010, vol./is. 23/5(366370), 14767058
Publication Date: 01 May 2010
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
79. Neonatal alloimmune thrombocytopenia caused by an antibody specific for a
newly identified allele of human platelet antigen-7.
Author(s): Koh Y, Taniue A, Ishii H, Matsuyama N, Amakishi E, Hayashi T, Furuta RA,
Fukumori Y, Hirayama F, Yoshimura K, Nagamine T, Tamai S, Nakano S
Citation: Transfusion, 01 June 2010, vol./is. 50/6(1276-1284), 00411132
Publication Date: 01 June 2010
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
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if required.
80. Intracranial hemorrhage in alloimmune thrombocytopenia: stratified
management to prevent recurrence in the subsequent affected fetus.
Author(s): Bussel JB, Berkowitz RL, Hung C, Kolb EA, Wissert M, Primiani A, Tsaur FW,
Macfarland JG
Citation: American Journal of Obstetrics & Gynecology, 01 August 2010, vol./is. 203/2(0-),
00029378
Publication Date: 01 August 2010
Abstract: OBJECTIVE: We sought to prevent intracranial hemorrhage (ICH) through
antenatal management of alloimmune thrombocytopenia. STUDY DESIGN: A total of 33
women (37 pregnancies) with alloimmune thrombocytopenia and ICH in a previous child
were stratified according to the timing of the previous child's ICH: extremely high risk (HR)
(n = 8) had ICH <28 weeks, very HR (n = 17) between 28-36 weeks, and HR (n = 12) in the
perinatal period. Treatment was initiated at 12 weeks with intravenous immunoglobulin 1
or 2 g/kg/wk, and if the fetal platelet count by cordocentesis was <30,000/mL despite
treatment, prednisone and/or more intravenous immunoglobulin were added. RESULTS:
Five of 37 fetuses suffered ICHs. Two ICHs had platelet counts >100,000/mL, and 1 was
grade I. The other 2 ICHs were unequivocal treatment failures; both were grade III-IV and
resulted in fetal demise. CONCLUSION: These findings demonstrate the success of
stratified treatment in these HR patients, which tailored interventions according to the
timing of the sibling's ICH.
Source: CINAHL
10
81. Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia:
systematic review.
Author(s): Kamphuis MM, Paridaans N, Porcelijn L, De Haas M, Van Der Schoot CE, Brand
A, Bonsel GJ, Oepkes D
Citation: BJOG: An International Journal of Obstetrics & Gynaecology, 01 October 2010,
vol./is. 117/11(1335-1343), 14700328
Publication Date: 01 October 2010
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
Available in fulltext at the ULHT Library and Knowledge Services' eJournal collection ;
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if required
82. Bilateral retinal detachments and preeclampsia: thrombotic thrombocytopenic
purpura or syndrome of haemolysis, elevated liver enzymes, low platelets?
Author(s): Mendez-Figueroa H, Davidson C
Citation: Journal of Maternal-Fetal & Neonatal Medicine, 01 October 2010, vol./is.
23/10(1268-1270), 14767058
Publication Date: 01 October 2010
Source: CINAHL
Full Text:
Available in fulltext at EBSCO Host
83. Clinical study on five cases of thrombotic thrombocytopenic purpura complicating
pregnancy.
Author(s): He Y, Chen Y, Zhao Y, Zhang Y, Yang W
Citation: Australian & New Zealand Journal of Obstetrics & Gynaecology, 01 December
2010, vol./is. 50/6(519-522), 00048666
Publication Date: 01 December 2010
Source: CINAHL
84. Established venous thromboembolism therapies: heparin, low molecular weight
heparins, and vitamin k antagonists, with a discussion of heparin-induced
thrombocytopenia.
Author(s): Pendleton RC, Rodgers GM, Hull RD
Citation: Clinics in Chest Medicine, 01 December 2010, vol./is. 31/4(691-706), 02725231
Publication Date: 01 December 2010
Abstract: For a majority of patients with venous thromboembolism (VTE), initial treatment
is straightforward and necessitates the immediate initiation of a parenteral anticoagulant
(eg, heparin or low molecular weight heparin), simultaneous initiation of long-term
therapy (eg, vitamin K antagonist), and discontinuation of the parenteral anticoagulant
after 5 days assuming that the vitamin K antagonist is therapeutic. This standardized
approach is based on numerous pivotal clinical trials completed over the past 3 decades.
11
Yet, advances in standardized VTE treatment continue to evolve and include issues related
to the selection and dosing of parenteral anticoagulants (eg, relative efficacy and dosing in
the obese patient, patients with renal impairment, and pregnant patients), optimal
location of initial care delivery, use of dosing initiation nomograms for vitamin K
antagonists with the potential of gene-based dosing, and demonstration that longterm
low molecular weight heparin therapy may be optimal for some patient populations (eg,
those with active cancer). Further, in parallel with the evolution of VTE treatment, there
have been remarkable advances in our understanding of heparin-induced
thrombocytopenia, a prothrombotic complication of parenteral anticoagulant use.
Source: CINAHL
85. Reversal of thrombocytopenia in a pregnant woman after changing
hemodiafiltration membranes... Am J Kidney Dis. 2010 Jun;55(6):e25-8.
Author(s): Venditto M, Bourry E, Szumilak D, Deray G
Citation: American Journal of Kidney Diseases, 01 March 2011, vol./is. 57/3(521-521),
02726386
Publication Date: 01 March 2011
Source: CINAHL
86. Immune thrombocytopenic purpura in pregnancy: a reappraisal of obstetric
management and outcome.
Author(s): Gasim, Turki
Citation: Journal of Reproductive Medicine, 01 March 2011, vol./is. 56/3-4(163-168),
00247758
Publication Date: 01 March 2011
Abstract: OBJECTIVE: To evaluate the complications of pregnancy and perinatal outcome
in women with idiopathic thrombocytopenic purpura (ITP). Study design: A retrospective
analysis of 38 singleton pregnancies, their course, obstetric management and perinatal
outcome of 32 patients with known ITP was undertaken. RESULTS: No major antenatal
complications were noted among the patients. There were no maternal deaths, and only 1
stillbirth occurred in the series. Fourteen infants were delivered by cesarean section and
24 by vaginal delivery. Neonatal cord blood platelet count was performed in each of the
live-born infants and revealed thrombocytopenia in 16 infants, but in only 6 (16.2%) of
them was the cord blood platelet count <50¥109/L. There was no neonatal death in the
study, although 6 infants required supportive treatment with corticosteroids and
intravenous immunoglobulin G. No maternal features could be used to predict the
neonatal platelet count at birth. These results are comparable with other studies in the
recent literature. CONCLUSION: Due to the low incidence of poor neonatal outcome in
mothers with ITP, obstetric intervention based solely on their platelet count is not
justified. Every patient with ITP should be managed individually, and the routine use of
cesarean section should be abandoned.
Source: CINAHL
87. Thrombotic thrombocytopenic purpura with complete molar pregnancy: a case
report.
Author(s): Meng, Hongmei, Kumar, Nirmala S., Nannapaneni, Jyothi, Inglis, Steven R.
Citation: Journal of Reproductive Medicine, 01 March 2011, vol./is. 56/3-4(169-171),
00247758
12
Publication Date: 01 March 2011
Abstract: BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is extremely rare.
This disease and its prompt diagnosis are important because TTP in pregnancy carries a
90% mortality rate. CASE: A 21-year-old woman underwent suction dilation and curettage
for molar pregnancy. Postoperatively the patient developed severe hypertension,
microangiopathic anemia, thrombocytopenia and chest pain associated with ischemic
cardiac changes. Despite blood and plasma transfusions and steroid therapy, the patient
continued to have worsening hemolysis and thrombocytopenia. TTP was diagnosed, and
plasmapheresis led to a rapid recovery. CONCLUSION: TTP can occur with molar
pregnancy. Making this diagnosis in a timely manner is crucial to ensure that potentially
life-saving plasmapheresis is initiated in a timely manner. To our knowledge, this is the
first reported case of thrombotic thrombocytopenic purpura with molar pregnancy.
Source: CINAHL
88. Thrombocytopenia in pregnancy.
Author(s): Bockenstedt PL
Citation: Hematology/Oncology Clinics of North America, 01 April 2011, vol./is. 25/2(293310), 08898588
Publication Date: 01 April 2011
Abstract: Thrombocytopenia in pregnancy is most frequently a benign process that does
not require intervention. However, 35% of cases of thrombocytopenia in pregnancy are
related to disease processes that may have serious bleeding consequences at delivery or
for which thrombocytopenia may be an indicator of a more severe systemic disorder
requiring emergent maternal and fetal care. Thus, all pregnant women with platelet
counts less than 100,000/[mu]L require careful hematological and obstetric consultation
to exclude more serious disorders.
Source: CINAHL
89. Microangiopathic disorders in pregnancy.
Author(s): Pels SG, Paidas MJ
Citation: Hematology/Oncology Clinics of North America, 01 April 2011, vol./is. 25/2(311322), 08898588
Publication Date: 01 April 2011
Abstract: Microangiopathic disorders present with thrombocytopenia, hemolytic anemia,
and multiorgan damage. In pregnancy, these disorders present a challenge both
diagnostically and therapeutically, with widely overlapping clinical scenarios and disparate
treatments. Although rare, a clear understanding of these diseases is important because
devastating maternal and fetal outcomes may ensue if there is misdiagnosis and improper
treatment. Microangiopathic disorders presenting in pregnancy are thus best assessed
and treated by both obstetric and hematology teams. As a better understanding of the
pathophysiology underlying each of the disease processes is gained, new diagnostic
testing and therapies will be available, which will lead to improved outcomes.
Source: CINAHL
90. Transfusion medicine and the pregnant patient.
Author(s): Lee AI, Kaufman RM
Citation: Hematology/Oncology Clinics of North America, 01 April 2011, vol./is. 25/2(39313
413), 08898588
Publication Date: 01 April 2011
Abstract: Alloimmunity in pregnancy is the basis for two of the major complications of
pregnancy in transfusion medicine: hemolytic disease of the fetus and newborn (HDFN),
and fetal and neonatal alloimmune thrombocytopenia (FNAIT). Use of Rh(D) immune
globulin has dramatically reduced the incidence of HDFN in Rh(D)-mismatched
pregnancies. Treatment of HDFN may involve intrauterine transfusion, with fetal and
neonatal survival rates of 70% to 90%. Treatments for FNAIT include immune globulin,
steroids, or in severe cases, intrauterine platelet transfusions. Transfusion medicine is
central to the management of pregnancy-associated complications such as postpartum
hemorrhage, parvovirus B19 infection, hemoglobinopathies, and aplastic anemia.
Source: CINAHL
91. Fetal alloimmune thrombocytopenia: is less invasive antenatal management safe?
Author(s): Mechoulan A, Kaplan C, Muller JY, Branger B, Philippe HJ, Oury JF, Ville Y, Winer
N
Citation: Journal of Maternal-Fetal & Neonatal Medicine, 01 April 2011, vol./is. 24/4(564567), 14767058
Publication Date: 01 April 2011
Source: CINAHL
92. Antenatal interventions for fetomaternal alloimmune thrombocytopenia.
Author(s): Rayment R, Brunskill SJ, Soothill PW, Roberts DJ, Bussel JB, Murphy MF
Citation: Cochrane Database of Systematic Reviews, 01 May 2011, vol./is. /5(0-),
1469493X
Publication Date: 01 May 2011
Abstract: Background:
Source: CINAHL
Full Text:
Available in fulltext at Wiley
MEDLINE Results
1. [Thrombocytopenia in pregnancy and neonatal outcomes].
Author(s): Chao S, Zeng CM, Liu J
Citation: Zhongguo Dangdai Erke Zazhi, October 2011, vol./is. 13/10(790-3), 10088830;1008-8830 (2011 Oct)
Publication Date: October 2011
Abstract: OBJECTIVE: To study the relationship between thrombocytopenia in pregnancy
associated with various causes and neonatal outcomes.METHODS: Medical records of 140
pregnant women with thrombocytopenia in pregnancy and the neonatal outcomes from
January 2009 to December 2010 were reviewed retrospectively. The pregnant women
were classified into four groups according to the causes of thrombocytopenia: gestational
thrombocytopenia (GT; n=94), pregnancy with immune thrombocytopenic purpura (ITP;
n=30), pregnancy with other hematological disease (aplastic anemia or myelodysplastic
14
syndrome; n=12), and other causes (n=4): pregnancy induced hypertension syndrome,
pregnancy with systemic lupus erythematosus, and pregnancy with alcoholic cirrhosis. The
neonatal outcomes in the four groups were compared.RESULTS: The premature birth
rates in the GT and the ITP groups were 11.3% and 16.7%, respectively. There was no
significant difference between the two groups. The premature birth rate in the other
hematological disease group was 53.8%, which was significantly higher than that in the GT
(P<0.01) and the ITP groups (P<0.05). Congenital passive immune thrombocytopenia was
found in 2 neonates (2%) in the GT group and in 4 neonates (13%) in the ITP group
(P<0.05). In addition, other diseases were also observed in neonates in the ITP group,
including 1 case (3%) of ITP and 1 case (3%) of Evans syndrome. Intracranial hemorrhage
occurred in one neonate (8%) in the other hematological disease group. Neonatal lupus
syndrome was found in 1 case (25%) in the other causes group.CONCLUSIONS:
Thrombocytopenia in pregnancy associated with different causes may result in different
neonatal outcomes.
Source: MEDLINE
2. Thrombocytopenia and disorders of platelet function in pregnancy.
Author(s): Kadir RA, McLintock C
Citation: Seminars in Thrombosis & Hemostasis, September 2011, vol./is. 37/6(640-52),
0094-6176;1098-9064 (2011 Sep)
Publication Date: September 2011
Abstract: Pregnancy is associated with physiological and pathological changes in platelet
numbers and function, which can be of clinical concern because of risks for maternal and
fetal or neonatal bleeding. Thrombocytopenia in pregnancy is frequently encountered and
may be due to increased platelet turnover and plasma dilution, immune-mediated
mechanisms, or a complication of a more severe underlying pregnancy-related disorder
such as preeclampsia. Inherited defects in platelet function and number may also manifest
during pregnancy with the risk of bleeding dependent on the underlying problem. In some
women, the diagnosis of thrombocytopenia will precede pregnancy but in others, the
problem is first identified when routine pregnancy blood tests are performed. An accurate
diagnosis and risk assessment in the antenatal period are essential for developing specific
plans for any antenatal interventions and for management of delivery and the postpartum
periods, and the neonate. Management of pregnant women with platelet disorders
requires a multidisciplinary approach and close collaboration between the obstetric and
hematology teams. Copyright Thieme Medical Publishers.
Source: MEDLINE
3. The pathophysiology of FNAIT cannot be deduced from highly selected
retrospective data.
Author(s): Kjeldsen-Kragh J, Husebekk A, Killie MK, Skogen B
Citation: Blood, September 2011, vol./is. 118/9(2638-9), 0006-4971;1528-0020 (2011 Sep
1)
Publication Date: September 2011
Source: MEDLINE
4. Prediction of fetal status in fetal/neonatal alloimmune thrombocytopenia (FNAIT)?.
Author(s): Sachs UJ, Bakchoul T, Kiefel V, Santoso S
Citation: Blood, September 2011, vol./is. 118/9(2637-8; author reply 2639-40), 000615
4971;1528-0020 (2011 Sep 1)
Publication Date: September 2011
Source: MEDLINE
5. A new platelet alloantigen, Swi(a) , located on glycoprotein Ia identified in a family
with fetal and neonatal alloimmune thrombocytopenia.
Author(s): Kroll H, Feldmann K, Zwingel C, Hoch J, Bald R, Bein G, Bayat B, Santoso S
Citation: Transfusion, August 2011, vol./is. 51/8(1745-54), 0041-1132;1537-2995 (2011
Aug)
Publication Date: August 2011
Abstract: BACKGROUND: Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a
bleeding disorder caused by transplacental passage of maternal antibodies to fetuses
whose platelets (PLTs) express the corresponding human PLT antigen (HPA). STUDY
DESIGNS AND METHODS: We observed a fetus with FNAIT who died from a severe
intracranial hemorrhage. Analysis of maternal serum in antigen capture assay with
paternal PLTs showed reactivity with PLT glycoprotein (GP)IIb/IIIa (alpha(IIb) beta(3) ) and
GPIa/IIa (alpha(2) beta(1) integrin), indicating the presence of anti-HPA-1a and an
additional alloantibody against GPIa (termed anti-Swi(a) ).RESULTS: By immunochemical
studies, the localization of the Swi(a) antigen on GPIa/IIa could be confirmed. Analysis of
paternal GPIa full-length cDNA showed a single-nucleotide substitution C(3347) T in Exon
28 resulting in a Thr(1087) Met amino acid substitution. Testing of family members by
polymerase chain reaction-restriction fragment length polymorphism using MslI
endonuclease showed perfect correlation with phenotyping. Extended family and
population studies showed that 4 of 10 members of the paternal family but none of 500
unrelated blood donors were Swi(a) carriers. Expression studies on allele-specific
transfected Chinese hamster ovary (CHO) cells confirmed that the single-amino-acid
substitution Thr(1087) Met was responsible for the formation of the Swi(a) epitope.
Adhesion of CHO cells expressing the Swi(a) alloantigen to immobilized collagens was not
impaired compared to the wild-type control and was not inhibited by anti-Swi(a)
alloantibodies.CONCLUSION: In this study we defined a new PLT alloantigen Swi(a) that
was involved in a case of additional immunization against HPA-1a. Our observations
demonstrate that combinations of PLT-specific alloantibodies may comprise lowfrequency alloantigens. Copyright 2011 American Association of Blood Banks.
Source: MEDLINE
Full Text:
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6. Fetal and neonatal alloimmune thrombocytopenia: prenatal interventions.
Author(s): Kamphuis MM, Oepkes D
Citation: Prenatal Diagnosis, July 2011, vol./is. 31/7(712-9), 0197-3851;1097-0223 (2011
Jul)
Publication Date: July 2011
Abstract: Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a potentially
devastating condition, which may lead to intracranial haemorrhage (ICH) in the fetus or
neonate, often with death or major neurological damage as consequence. In the absence
of screening, preventive measures are only possible in the next pregnancy of women with
16
an affected child. Controversy exists on the best intervention to minimise the risk of ICH.
Most centres have abandoned treatment with serial fetal blood sampling (FBS) and
platelet transfusions, because of a high rate of complications and the availability of quite
effective non-invasive alternatives. In pregnancies with FNAIT and a previous affected
child without ICH, weekly intravenous administration of immunoglobulins to the mother
appears close to 100% effective to prevent fetal or neonatal ICH. Some centres add
prednisone; this combination leads to slightly higher platelet counts at birth. In pregnant
women with a previous child with ICH, the recurrence risk seems particularly high, and
more aggressive maternal medical treatment is recommended, starting earlier with
immunoglobulins. Whether a higher intravenous immunoglobulin dose or the addition of
prednisone is really necessary is unclear. What does seem to be clear is that the use of FBS
should be minimised, possibly even abandoned completely. Copyright Copyright 2011
John Wiley & Sons, Ltd.
Source: MEDLINE
7. Exacerbation of thrombocytopenia in a pregnant woman with thrombocytopeniaabsent radius syndrome.
Author(s): Bot-Robin V, Vaast P, Deruelle P
Citation: International Journal of Gynaecology & Obstetrics, July 2011, vol./is. 114/1(77-8),
0020-7292;1879-3479 (2011 Jul)
Publication Date: July 2011
Source: MEDLINE
8. Successful management of a planned pregnancy in severe congenital thrombotic
thrombocytopaenic purpura: the Upshaw-Schulman syndrome.
Author(s): Richter J, Strandberg K, Lindblom A, Strevens H, Karpman D, Wide-Swensson D
Citation: Transfusion Medicine, June 2011, vol./is. 21/3(211-3), 0958-7578;1365-3148
(2011 Jun)
Publication Date: June 2011
Source: MEDLINE
9. A founder mutation in the MPL gene causes congenital amegakaryocytic
thrombocytopenia (CAMT) in the Ashkenazi Jewish population.
Author(s): Jalas C, Anderson SL, Laufer T, Martimucci K, Bulanov A, Xie X, Ekstein J, Rubin
BY
Citation: Blood Cells Molecules & Diseases, June 2011, vol./is. 47/1(79-83), 10799796;1096-0961 (2011 Jun 15)
Publication Date: June 2011
Abstract: Congenital amegakaryocytic thrombocytopenia (MIM #604498) (CAMT) is a rare
inherited disease presenting as severe thrombocytopenia in infancy. Untreated, many
CAMT patients develop aplastic anemia within the first decade of life; the only effective
treatment of CAMT is bone marrow transplantation. CAMT is the result of the presence of
homozygous or compound heterozygous mutations in the thrombopoietin receptorencoding gene, MPL. We report here the identification and characterization of a founder
mutation in MPL in the Ashkenazi Jewish (AJ) population. This mutation, termed
c.79+2T>A, is a T to A transversion in the invariant second base of the intron 1 donor
splice site. Analysis of a random sample of 2018 individuals of AJ descent revealed a
carrier frequency of approximately 1 in 75. Genotyping of six loci adjacent to the MPL
17
gene in the proband and in the 27 individuals identified as carriers of the c.79+2T>A
mutation revealed that the presence of this mutation in the AJ population is due to a
single founder. The observed carrier frequency predicts an incidence of CAMT in the AJ
population of approximately 1 in 22,500 pregnancies. The identification of this mutation
will enable population carrier testing and will facilitate the identification and treatment of
individuals homozygous for this mutation. Copyright Copyright 2011 Elsevier Inc. All rights
reserved.
Source: MEDLINE
10. Fetomaternal alloimmune thrombocytopenia: increasing awareness.
Author(s): Dickinson JE
Citation: Australian & New Zealand Journal of Obstetrics & Gynaecology, June 2011,
vol./is. 51/3(189-90), 0004-8666;1479-828X (2011 Jun)
Publication Date: June 2011
Source: MEDLINE
11. Management of severe immune thrombocytopenic purpura in a pregnant woman
with inevitable preterm forceps breech delivery.
Author(s): Cho FN
Citation: Taiwanese Journal of Obstetrics & Gynecology, June 2011, vol./is. 50/2(227-9),
1028-4559;1875-6263 (2011 Jun)
Publication Date: June 2011
Source: MEDLINE
12. [Advance of clinical study on immune thrombocytopenia caused by irregular
antibodies].
Author(s): Zhu LL, Zhang CG
Citation: Zhongguo Shi Yan Xue Ye Xue Za Zhi, June 2011, vol./is. 19/3(839-42), 10092137;1009-2137 (2011 Jun)
Publication Date: June 2011
Abstract: The platelet antibodies mainly include platelet-specific and related antibodies,
which belong to irregular antibodices. They are produced by autoimmune, drug-induced
or isoimmunization (such as pregnancy, blood transfusion and so on), the irregular IgG
and/or IgM antibodies produce and lead to platelet transfusion refractoriness (PTR), posttransfusion purpura (PTP) and isoimmune neonatal throbocytopenic purpura (INTP),
idiopathic thrombocytopenic purpura and so on. It is very necessary to screen and identify
the irregular antibodies before blood transfusion or parturition. Except some serological
detections should be done first, flow cytometry and molecular biological techniques such
as PCR and PCR-SSP are applied to detect the difficult-matching patients' genotypes and
fetal genotypes in order to further predict fetal INTP and to provide the right blood for
difficult-matching patients, therefore, some measures must be taken early for prevention
and treatment of immune thrombocytopenic purpura. In this review, the production,
typing and laboratory tests of irregular antibodies, as well as the pathogenesis and clinical
symptoms of diseases caused by irregular antibodies, and the current progress are
summarized.
Source: MEDLINE
13. Use of fondaparinux in a pregnant woman with pulmonary embolism and heparin18
induced thrombocytopenia.
Author(s): Ciurzynski M, Jankowski K, Pietrzak B, Mazanowska N, Rzewuska E, Kowalik R,
Pruszczyk P
Citation: Medical Science Monitor, May 2011, vol./is. 17/5(CS56-9), 1234-1010;1643-3750
(2011 May)
Publication Date: May 2011
Abstract: BACKGROUND: A serious complication of heparin treatment, heparin-induced
thrombocytopenia (HIT) is rarely observed in pregnant women. Drug therapy during
pregnancy should always be chosen to minimize fetal risk. The management of HIT in
pregnancy represents a medical challenge. Unlike heparins, the anticoagulants used in
patients with HIT do cross the placenta, with unknown fetal effects.CASE REPORT: We
present a case of a 24-year-old female presenting for care at 34 weeks of gestation with
acute pulmonary embolism treated initially with unfractionated heparin (UFH) and low
molecular weight heparin (LMWH), who developed HIT. She was then successfully treated
with fondaparinux.CONCLUSIONS: To the best of our knowledge, this is one of the first
case reports describing a successful use of fondaparinux in the treatment of HIT in a thirdtrimester pregnant woman, providing a novel approach for this subset of patients.
Source: MEDLINE
14. Microangiopathic disorders in pregnancy.
Author(s): Pels SG, Paidas MJ
Citation: Hematology - Oncology Clinics of North America, April 2011, vol./is. 25/2(311-22,
viii), 0889-8588;1558-1977 (2011 Apr)
Publication Date: April 2011
Abstract: Microangiopathic disorders present with thrombocytopenia, hemolytic anemia,
and multiorgan damage. In pregnancy, these disorders present a challenge both
diagnostically and therapeutically, with widely overlapping clinical scenarios and disparate
treatments. Although rare, a clear understanding of these diseases is important because
devastating maternal and fetal outcomes may ensue if there is misdiagnosis and improper
treatment. Microangiopathic disorders presenting in pregnancy are thus best assessed
and treated by both obstetric and hematology teams. As a better understanding of the
pathophysiology underlying each of the disease processes is gained, new diagnostic
testing and therapies will be available, which will lead to improved outcomes. Copyright
Copyright 2011 Elsevier Inc. All rights reserved.
Source: MEDLINE
15. Thrombocytopenia in pregnancy.
Author(s): Bockenstedt PL
Citation: Hematology - Oncology Clinics of North America, April 2011, vol./is. 25/2(293310, vii-viii), 0889-8588;1558-1977 (2011 Apr)
Publication Date: April 2011
Abstract: Thrombocytopenia in pregnancy is most frequently a benign process that does
not require intervention. However, 35% of cases of thrombocytopenia in pregnancy are
related to disease processes that may have serious bleeding consequences at delivery or
for which thrombocytopenia may be an indicator of a more severe systemic disorder
requiring emergent maternal and fetal care. Thus, all pregnant women with platelet
counts less than 100,000/muL require careful hematological and obstetric consultation to
19
exclude more serious disorders. Copyright Copyright 2011 Elsevier Inc. All rights reserved.
Source: MEDLINE
16. Fetal alloimmune thrombocytopenia: is less invasive antenatal management
safe?.
Author(s): Mechoulan A, Kaplan C, Muller JY, Branger B, Philippe HJ, Oury JF, Ville Y, Winer
N, French GROG
Citation: Journal of Maternal-Fetal & Neonatal Medicine, April 2011, vol./is. 24/4(564-7),
1476-4954;1476-4954 (2011 Apr)
Publication Date: April 2011
Abstract: OBJECTIVES: The aim of this study was to review recent multicenter data on
antenatal management of anti-HPA-1a fetal alloimmune thrombocytopenia and, based on
this retrospective study and on recent literature, to evaluate if FBS modified the
obstetrical management.MATERIAL AND METHODS: This retrospective study in France
includes 23 pregnancies in 21 women who had a previous thrombocytopenic infant due to
anti HPA-1a alloimmunization. All pregnant women received intravenous immunoglobulin
(IVIG) treatment, with or without corticosteroids. Fetal blood sampling (FBS) was
performed before any therapy (four cases) or during pregnancy (nine cases).RESULTS:
Infants whose mother received treatment had a significantly higher neonatal platelet
count than the corresponding sibling (p = 0.003). In eight cases, therapy was started late
during pregnancy. In three cases, treatment was discontinued 3 or 4 weeks before birth,
and this was associated with a poorer result. No in utero intracranial hemorrhage was
recorded in the infants for whom maternal therapy continued to term. Adverse effects
were not observed in any case. All babies were delivered by cesarean even when FBS was
performed. One emergency cesarean was performed for fetal bradycardia after
FBS.CONCLUSION: This study confirmed that maternal therapy with intravenous
immunoglobulin for fetal alloimmune thrombocytopenia gives satisfactory results. It also
showed that a less invasive approach, especially a reduction in the number of fetal blood
samples, is possible without deleterious consequences. This observation suggests also to
start IVIG early during pregnancy and to continue treatment up to delivery.
Source: MEDLINE
17. Life-threatening postpartum hemolysis, elevated liver functions tests, low
platelets syndrome versus thrombocytopenic purpura - Therapeutic plasma exchange is
the answer.
Author(s): Nasa P, Dua JM, Kansal S, Chadha G, Chawla R, Manchanda M
Citation: Indian Journal of Critical Care Medicine, April 2011, vol./is. 15/2(126-9), 09725229;1998-359X (2011 Apr)
Publication Date: April 2011
Abstract: The differential diagnosis of life-threatening microangiopathic disorders in a
postpartum female includes severe preeclampsia-eclampsia, hemolysis, elevated liver
functions tests, low platelets syndrome and thrombotic thrombocytopenic purpura. There
is considerable overlapping in the clinical and laboratory findings between these
conditions, and hence an exact diagnosis may not be always possible. However, there is
considerable maternal mortality and morbidity associated with these disorders. This case
underlines the complexity of pregnancy-related microangiopathies regarding their
differential diagnosis, multiple organ dysfunction and role of therapeutic plasma exchange
in their management.
Source: MEDLINE
20
Full Text:
Available in fulltext at National Library of Medicine
18. Reversal of thrombocytopenia in a pregnant woman after changing
hemodiafiltration membranes.
Author(s): Venditto M, Bourry E, Szumilak D, Deray G
Citation: American Journal of Kidney Diseases, March 2011, vol./is. 57/3(521), 02726386;1523-6838 (2011 Mar)
Publication Date: March 2011
Source: MEDLINE
19. Prediction of the fetal status in noninvasive management of alloimmune
thrombocytopenia.
Author(s): Bertrand G, Drame M, Martageix C, Kaplan C
Citation: Blood, March 2011, vol./is. 117/11(3209-13), 0006-4971;1528-0020 (2011 Mar
17)
Publication Date: March 2011
Abstract: Fetal/neonatal alloimmune thrombocytopenia is the most common cause of
severe thrombocytopenia in the fetus and in an otherwise healthy newborn. To counter
the consequences of severe fetal thrombocytopenia, antenatal therapies have been
implemented. Predictive parameters for fetal severe thrombocytopenia are important for
the development of noninvasive strategy and tailored intervention. We report here data
concerning 239 pregnancies in 75 HPA-1bb women. Analysis of the index cases (diagnosis
of fetal/neonatal alloimmune thrombocytopenia) did not show any significant correlation
between the severity of the disease and the maternal genetic background (ABO blood
group and HLA-DRB3 allele). Subsequent pregnancies were managed, and therapy
effectiveness was evaluated. The highest mean newborn platelet count was observed for
a combination of intravenous immunoglobulin and steroids (135 x 109/L; 54 newborns)
compared with intravenous immunoglobulin alone (89 x 109/L; 27 newborns). The
maternal anti-HPA-1a antibody concentration measured before any treatment and before
28 weeks of gestation was predictive of the fetal status. The weighted areas under curves
of the maternal alloantibody concentrations were predictive of therapy response. To
conclude, this large retrospective survey gives new insights on maternal predictive
parameters for fetal status and therapy effectiveness allowing noninvasive strategies.
Source: MEDLINE
Full Text:
Available in fulltext at Highwire Press
20. Thrombotic thrombocytopenic purpura with complete molar pregnancy: a case
report.
Author(s): Meng H, Kumar NS, Nannapaneni J, Inglis SR
Citation: Journal of Reproductive Medicine, March 2011, vol./is. 56/3-4(169-71), 00247758;0024-7758 (2011 Mar-Apr)
Publication Date: March 2011
Abstract: BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is extremely rare.
This disease and its prompt diagnosis are important because TTP in pregnancy carries a
90% mortality rate.CASE: A 21-year-old woman underwent suction dilation and curettage
21
for molar pregnancy. Postoperatively the patient developed severe hypertension,
microangiopathic anemia, thrombocytopenia and chest pain associated with ischemic
cardiac changes. Despite blood and plasma transfusions and steroid therapy, the patient
continued to have worsening hemolysis and thrombocytopenia. TTP was diagnosed, and
plasmapheresis led to a rapid recovery.CONCLUSION: TTP can occur with molar
pregnancy. Making this diagnosis in a timely manner is crucial to ensure that potentially
life-saving plasmapheresis is initiated in a timely manner. To our knowledge, this is the
first reported case of thrombotic thrombocytopenic purpura with molar pregnancy.
Source: MEDLINE
21. Immune thrombocytopenic purpura in pregnancy: a reappraisal of obstetric
management and outcome.
Author(s): Gasim T
Citation: Journal of Reproductive Medicine, March 2011, vol./is. 56/3-4(163-8), 00247758;0024-7758 (2011 Mar-Apr)
Publication Date: March 2011
Abstract: OBJECTIVE: To evaluate the complications of pregnancy and perinatal outcome
in women with idiopathic thrombocytopenic purpura (ITP).STUDY DESIGN: A retrospective
analysis of 38 singleton pregnancies, their course, obstetric management and perinatal
outcome of 32 patients with known ITP was undertaken.RESULTS: No major antenatal
complications were noted among the patients. There were no maternal deaths, and only 1
stillbirth occurred in the series. Fourteen infants were delivered by cesarean section and
24 by vaginal delivery. Neonatal cord blood platelet count was performed in each of the
live-born infants and revealed thrombocytopenia in 16 infants, but in only 6 (16.2%) of
them was the cord blood platelet count < 50 x 10(9)/L. There was no neonatal death in the
study, although 6 infants required supportive treatment with corticosteroids and
intravenous immunoglobulin G. No maternal features could be used to predict the
neonatal platelet count at birth. These results are comparable with other studies in the
recent literature.CONCLUSION: Due to the low incidence of poor neonatal outcome in
mothers with ITP, obstetric intervention based solely on their platelet count is not
justified. Every patient with ITP should be managed individually, and the routine use of
cesarean section should be abandoned.
Source: MEDLINE
22. TAR syndrome and esophagial atresia: a concomitant or variant condition?.
Author(s): Peker E, Cagan E, Dogan M, Sal E, Kirimi E
Citation: Journal of Maternal-Fetal & Neonatal Medicine, February 2011, vol./is. 24/2(2268), 1476-4954;1476-4954 (2011 Feb)
Publication Date: February 2011
Abstract: Thrombocytopenia with absent radii (TAR) is rare cause of neonatal
thrombocytopenia. TAR syndrome and esophageal atresia with tracheoesophageal fistula
has been reported in only two cases in literature. Our case was the first in literature with
unilateral TAR syndrome and bilateral absence of thumbs accompanying EA.
Source: MEDLINE
23. Fetal and neonatal alloimmune thrombocytopenia: harvesting the evidence to
develop a clinical approach to management.
Author(s): Symington A, Paes B
22
Citation: American Journal of Perinatology, February 2011, vol./is. 28/2(137-44), 07351631;1098-8785 (2011 Feb)
Publication Date: February 2011
Abstract: Neonatal alloimmune thrombocytopenia (NAIT) is the most common cause of
severe thrombocytopenia in an otherwise healthy newborn. The most serious
complication is intracranial hemorrhage, which can occur either in the fetus or the
newborn. Despite the known serious sequelae, both antenatal management and neonatal
treatment modalities are plagued by the lack of gold standard evidence to appropriately
direct therapy. Maternal, risk-based therapeutic approaches range from invasive protocols
to relatively benign noninvasive strategies to avoid serious procedural complications.
Intravenous immunoglobulin (IVIG) with or without steroids and fetal blood sampling
constitute the mainstay of antenatal management. Neonatal interventions principally
focus on the use of antigen-negative compatible or random donor platelets and IVIG.
While awaiting the results of controlled trials, each institution must develop a
standardized, collaborative, multidisciplinary approach to the screening, diagnostic
evaluation, and management of unexpected and anticipated NAIT based on experience,
product availability, and emerging scientific data. Copyright Thieme Medical Publishers.
Source: MEDLINE
24. The incidence and outcomes of fetomaternal alloimmune thrombocytopenia: a
UK national study using three data sources.
Author(s): Knight M, Pierce M, Allen D, Kurinczuk JJ, Spark P, Roberts DJ, Murphy MF
Citation: British Journal of Haematology, February 2011, vol./is. 152/4(460-8), 00071048;1365-2141 (2011 Feb)
Publication Date: February 2011
Abstract: Fetomaternal alloimmune thrombocytopenia (FMAIT) is the most common
cause of severe neonatal thrombocytopenia in otherwise well, term infants. First
pregnancies are often severely affected. This descriptive, population-based national study
was undertaken in order to inform the case for antenatal screening. Cases were identified
using three sources and capture-recapture techniques used to generate a robust
incidence estimate. One hundred and seventy three cases were identified between
October 2006 and September 2008. An extra 20 cases were estimated from capturerecapture analysis, giving an estimated incidence of clinically detected FMAIT of 12.4
cases per 100[em space]000 total births (95%confidence interval: 10.7, 14.3). Fifty-two
cases (30%) were known at the start of pregnancy; 120 (70%) were unknown (n=115) or
unrecognized (n=5). Unknown cases were more likely to experience a haemorrhagic
complication (67% vs. 5%) (P<0.001) and more likely to have an intracranial haemorrhage
(20% vs. 4%) (P=0.014) than known cases receiving antenatal management. In view of the
incidence of severe disease identified, further assessment of the case for antenatal
screening is important. There were a number of cases in which the significance of a history
of FMAIT in a previous sibling was not recognized and there is a need to raise awareness
of the importance of this diagnosis. Copyright 2011 Blackwell Publishing Ltd.
Source: MEDLINE
25. Advances in ITP - Therapy and Quality of Life - A Patient Survey.
Author(s): Matzdorff AC, Arnold G, Salama A, Ostermann H, Eberle S, Hummler S
Citation: PLoS ONE [Electronic Resource], 2011, vol./is. 6/11(e27350), 1932-6203;19326203 (2011)
Publication Date: 2011
23
Abstract: BACKGROUND: Current guidelines recommend glucocorticoids and splenectomy
as standard 1(st) and 2(nd) line treatments for chronic immune thrombocytopenia (ITP).
We sought to find out how German ITP-patients are treated with respect to these
guidelines.METHODS: Members of a patient support association >=18 years with a selfreported history of chronic ITP>12 months were surveyed with a web-based
questionnaire.RESULTS: 122 questionnaires were evaluated. 70% of patients had chronic
ITP for more than 5 years and 20% an average platelet count of <=30.10(9)/L. 41% of the
patients reported haematomas or petechiae more than once or twice and up to 12 times
or more per year and 17% oropharyngeal and nasal bleeds. 11% had been admitted to
hospital during the last 12 months. 88% had received or currently receive glucocorticoids,
27% were splenectomised. IVIG had been given to 55%, rituximab to 22%, anti-D to 12%,
ciclosporin to 7%, while complementary and alternative medical treatments had been
used by 36%. 50 women responded to questions concerning pregnancy. 14 (28%) had
been advised not to become pregnant. 23 reported pregnancies and 10 (44%) required
treatment for their ITP during pregnancy.CONCLUSION: Glucocorticoids are the most
common therapy for chronic ITP but complementary and alternative treatments already
come second and less than 3-Jan of patients are splenectomised. This and the frequent
use of complementary medicines suggests patients' dissatisfaction with conventional
approaches. Many patients receive off-label therapies. There is a major need for adequate
counselling and care for pregnant ITP-patients.
Source: MEDLINE
Full Text:
Available in fulltext at National Library of Medicine
26. Antenatal interventions for fetomaternal alloimmune thrombocytopenia.
Author(s): Rayment R, Brunskill SJ, Soothill PW, Roberts DJ, Bussel JB, Murphy MF
Citation: Cochrane Database of Systematic Reviews, 2011, vol./is. /5(CD004226), 13616137;1469-493X (2011)
Publication Date: 2011
Abstract: BACKGROUND: Fetomaternal alloimmune thrombocytopenia results from the
formation of antibodies by the mother which are directed against a fetal platelet
alloantigen inherited from the father. The resulting fetal thrombocytopenia (reduced
platelet numbers) may cause bleeding, particularly into the brain, before or shortly after
birth. Antenatal treatment of fetomaternal alloimmune thrombocytopenia includes the
administration of intravenous immunoglobulin (IVIG) and/or corticosteroids to the mother
to prevent severe fetal thrombocytopenia. IVIG and corticosteroids both have short-term
and possibly long-term side effects. IVIG is also costly and optimal regimens need to be
identified.OBJECTIVES: To determine the optimal antenatal treatment of fetomaternal
alloimmune thrombocytopenia to prevent fetal and neonatal haemorrhage and
death.SEARCH STRATEGY: We searched the Cochrane Pregnancy and Childbirth Group's
Trials Register (28 February 2011) and bibliographies of relevant publications and review
articles.SELECTION CRITERIA: Randomised controlled studies comparing any intervention
with no treatment, or comparing any two interventions.DATA COLLECTION AND ANALYSIS:
Two review authors independently assessed eligibility, trial quality and extracted
data.MAIN RESULTS: We included four trials involving 206 people. One trial involving 39
people compared a corticosteroid (prednisone) versus IVIG alone. In this trial, where
analysable data were available, there was no statistically significant differences between
the treatment arms for predefined outcomes. Three trials involving 167 people compared
IVIG plus a corticosteroid (prednisone in two trials and dexamethasone in one trial) versus
IVIG alone. In these trials there was no statistically significant difference in the findings
between the treatment arms for predefined outcomes (intracranial haemorrhage; platelet
24
count at birth and preterm birth). Lack of complete data sets and important differences in
interventions precluded the pooling of data from these trials.AUTHORS' CONCLUSIONS:
The optimal management of fetomaternal alloimmune thrombocytopenia remains
unclear. Lack of complete data sets for two trials and differences in interventions
precluded the pooling of data from these trials which may have enabled a more
developed analysis of the trial findings. Further trials would be required to determine
optimal treatment (the specific medication and its dose and schedule). Such studies
should include long-term follow up of all children and mothers.
Source: MEDLINE
Full Text:
Available in fulltext at Wiley
27. Rituximab for management of refractory pregnancy-associated immune
thrombocytopenic purpura.
Author(s): Gall B, Yee A, Berry B, Birchman D, Hayashi A, Dansereau J, Hart J
Citation: Journal of Obstetrics & Gynaecology Canada: JOGC, December 2010, vol./is.
32/12(1167-71), 1701-2163;1701-2163 (2010 Dec)
Publication Date: December 2010
Abstract: BACKGROUND: Rituximab is a novel therapy for immune thrombocytopenic
purpura (ITP); however, information about its safety in pregnancy is limited. This case
illustrates the successful use of rituximab to treat pregnancy-associated ITP.CASE: A 34year-old woman presented with severe ITP at 23 weeks' gestation. Standard treatment
with corticosteroids, intravenous immune globulin, and splenectomy failed to raise the
platelet count. Due to ongoing bleeding, rituximab was given in the 26th week of
pregnancy. The platelet count rose to over 100 x 10(9)/L after four weeks. The neonatal Blymphocyte count normalized at four months after delivery. There were no neonatal
complications of rituximab therapy.CONCLUSION: Rituximab may be safe for use in
treating pregnancy-associated ITP. This case highlights the need to investigate further the
safety and efficacy of rituximab in pregnancy.
Source: MEDLINE
28. Clinical study on five cases of thrombotic thrombocytopenic purpura complicating
pregnancy.
Author(s): He Y, Chen Y, Zhao Y, Zhang Y, Yang W
Citation: Australian & New Zealand Journal of Obstetrics & Gynaecology, December 2010,
vol./is. 50/6(519-22), 0004-8666;1479-828X (2010 Dec)
Publication Date: December 2010
Abstract: OBJECTIVE: The aim of this study was to investigate the potential role of
ADAMTS13 analysis in the early recognition and management of thrombotic
thrombocytopenic purpura (TTP) in pregnant women.METHODS: Five cases of TTP were
evaluated retrospectively. Clinical and laboratory findings, von Willebrand factor (vWF)cleaving metalloprotease (ADAMTS13) activity and maternal and neonatal outcome were
recorded and analysed.RESULTS: Five cases were all nulliparous. ADAMTS13 assay was
performed and the enzyme activity was less than 5% of the normal controls in three cases.
Gene mutation in the 9th exon resulting in amino acid exchange 349Arg->Cys in
ADAMTS13 was identified in one patient. After treatment including transfusion of freshfrozen plasma (n = 5), packed red blood cells (n = 5), platelet transfusions (n = 2) and/or
continued renal replacement therapy (CRRT) (n = 1) and plasma exchange (n = 2), three
patients were alive, one died on postpartum day 6 in hospital without plasma exchange
25
and one of familial TTP died three months after discharge.CONCLUSION: With increasing
awareness, extra-attention must be paid to patients with thrombotic microangiopathy
and to measurement of ADAMTS13 activity for early diagnosis. Although severe
ADAMTS13 deficiency may be helpful for TTP, it may not be sensitive enough to identify
all TTP patients. Therefore, despite ADAMTS13 result positive or negative, prompt
aggressive management should include early termination of pregnancy, plasma
transfusion and/or plasma exchange. Copyright 2010 The Authors. Australian and New
Zealand Journal of Obstetrics and Gynaecology Copyright 2010 The Royal Australian and
New Zealand College of Obstetricians and Gynaecologists.
Source: MEDLINE
29. Established venous thromboembolism therapies: heparin, low molecular weight
heparins, and vitamin K antagonists, with a discussion of heparin-induced
thrombocytopenia.
Author(s): Pendleton RC, Rodgers GM, Hull RD
Citation: Clinics in Chest Medicine, December 2010, vol./is. 31/4(691-706), 02725231;1557-8216 (2010 Dec)
Publication Date: December 2010
Abstract: For a majority of patients with venous thromboembolism (VTE), initial treatment
is straightforward and necessitates the immediate initiation of a parenteral anticoagulant
(eg, heparin or low molecular weight heparin), simultaneous initiation of long-term
therapy (eg, vitamin K antagonist), and discontinuation of the parenteral anticoagulant
after 5 days assuming that the vitamin K antagonist is therapeutic. This standardized
approach is based on numerous pivotal clinical trials completed over the past 3 decades.
Yet, advances in standardized VTE treatment continue to evolve and include issues related
to the selection and dosing of parenteral anticoagulants (eg, relative efficacy and dosing in
the obese patient, patients with renal impairment, and pregnant patients), optimal
location of initial care delivery, use of dosing initiation nomograms for vitamin K
antagonists with the potential of gene-based dosing, and demonstration that longterm
low molecular weight heparin therapy may be optimal for some patient populations (eg,
those with active cancer). Further, in parallel with the evolution of VTE treatment, there
have been remarkable advances in our understanding of heparin-induced
thrombocytopenia, a prothrombotic complication of parenteral anticoagulant use.
Copyright Copyright 2010 Elsevier Inc. All rights reserved.
Source: MEDLINE
30. Fetal genotyping for platelets antigens: a precise tool for alloimmune
thrombocytopenia: case report and literature review.
Author(s): Nomura ML, Couto E, Martinelli BM, Barjas-Castro ML, Barini R, Passini Junior R,
Castro V
Citation: Archives of Gynecology & Obstetrics, November 2010, vol./is. 282/5(573-5),
0932-0067;1432-0711 (2010 Nov)
Publication Date: November 2010
Abstract: INTRODUCTION: Maternal-fetal alloimmune thrombocytopenia complicates
about 0.1% of all pregnancies and is associated with major fetal and neonatal morbidity
and mortality, especially spontaneous central nervous system bleeding leading to death
and neurological handicaps. Successful prevention and treatment depend on the
identification of at-risk possible carriers of anti-platelet antibodies.CASE REPORT: We
report a case of a mother with a previous child that developed neonatal hemorrhage;
HPA-5b anti-platelet antibodies were detected post-natally. During the next pregnancy,
26
fetal genotyping confirmed the presence of HPA-5b antigen; she was treated with weekly
intravenous human immunoglobulin and oral prednisone. Pregnancy evolved without
remarkable features and a full-term baby was delivered, with normal platelet
counts.CONCLUSION: Fetal alloimmune thrombocytopenia is a potentially lethal condition,
but early detection and prevention lead to successful outcome in most cases.
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
31. Antenatal treatment of fetal alloimmune thrombocytopenia: a current
perspective.
Author(s): Vinograd CA, Bussel JB
Citation: Haematologica, November 2010, vol./is. 95/11(1807-11), 0390-6078;1592-8721
(2010 Nov)
Publication Date: November 2010
Source: MEDLINE
Full Text:
Available in fulltext at National Library of Medicine
Available in fulltext at Highwire Press
32. Fetal alloimmune thrombocytopenia and maternal intravenous immunoglobulin
infusion.
Author(s): Giers G, Wenzel F, Stockschlader M, Riethmacher R, Lorenz H, Tutschek B
Citation: Haematologica, November 2010, vol./is. 95/11(1921-6), 0390-6078;1592-8721
(2010 Nov)
Publication Date: November 2010
Abstract: BACKGROUND: Different therapeutic approaches have been used in fetalneonatal alloimmune thrombocytopenia, but many centers administer immunoglobulin G
infusions to the pregnant woman. We studied the effect of maternal antenatal
immunoglobulin infusions on fetal platelet counts in pregnancies with fetal alloimmune
thrombocytopenia.DESIGN AND METHODS: We retrospectively analyzed the clinical
courses of fetuses with fetal alloimmune thrombocytopenia whose mothers were treated
with immunoglobulin G infusions in a single center between 1999 and 2005. In a centerspecific protocol, weekly maternal immunoglobulin G infusions were given to 25 pregnant
women with previously affected neonates and four women with strong platelet
antibodies, but no previous history of fetal alloimmune thrombocytopenia; before each
infusion diagnostic fetal blood sampling was performed to determine fetal platelet counts
and immunoglobulin G levels.RESULTS: There were 30 fetuses with fetal alloimmune
thrombocytopenia, confirmed by initial fetal blood sampling showing fetal platelet counts
between 4x10(9)/L and 130x10(9)/L and antibody-coated fetal platelets using a
glycoprotein specific assay. Despite weekly antenatal maternal immunoglobulin G
infusions fetal platelet counts did not change significantly. Maternal and fetal
immunoglobulin G levels, measured before every infusion, increased significantly with the
number of maternal immunoglobulin G infusions.CONCLUSIONS: In this group of fetuses
with fetal alloimmune thrombocytopenia no consistent increase of fetal platelets was
achieved as a result of regular maternal immunoglobulin G infusions.
Source: MEDLINE
27
Full Text:
Available in fulltext at National Library of Medicine
Available in fulltext at Highwire Press
33. Pulmonary embolectomy in heparin-induced thrombocytopenia and thrombosis?
Safety of heparin use.
Author(s): Sachithanandan A
Citation: Interactive Cardiovascular & Thoracic Surgery, November 2010, vol./is.
11/5(681), 1569-9285;1569-9285 (2010 Nov)
Publication Date: November 2010
Source: MEDLINE
Full Text:
Available in fulltext at Highwire Press
34. Successful surgical management of massive pulmonary embolism during the
second trimester in a parturient with heparin-induced thrombocytopenia.
Author(s): Hajj-Chahine J, Jayle C, Tomasi J, Corbi P
Citation: Interactive Cardiovascular & Thoracic Surgery, November 2010, vol./is. 11/5(67981), 1569-9285;1569-9285 (2010 Nov)
Publication Date: November 2010
Abstract: Cardiopulmonary bypass during pregnancy is associated with a high fetal and
maternal mortality. We report a successful pulmonary embolectomy in a woman at the
27th week of pregnancy; we performed surgical pulmonary embolectomy under
cardiopulmonary bypass to restore adequate hemodynamic stability and to relieve right
ventricle strain. We discuss the decision made for the preferred anticoagulation drug in
the setting of heparin-induced thrombocytopenia in the gravida. The pregnancy was
carried to term and she delivered a healthy boy at 38[NON-BREAKING SPACE]weeks of
gestation.
Source: MEDLINE
Full Text:
Available in fulltext at Highwire Press
35. Repeated intrauterine IgG infusions in foetal alloimmune thrombocytopenia do
not increase foetal platelet counts.
Author(s): Giers G, Wenzel F, Riethmacher R, Lorenz H, Tutschek B
Citation: Vox Sanguinis, November 2010, vol./is. 99/4(348-53), 0042-9007;1423-0410
(2010 Nov)
Publication Date: November 2010
Abstract: BACKGROUND AND OBJECTIVES: Foetal alloimmune thrombocytopenia (FNAIT)
is often treated transplacentally with maternally administered i.v. immunoglobulins, but
not all foetuses show a consistent platelet increase during such treatment.MATERIALS
AND METHODS: We retrospectively analysed data from a cohort of ten foetuses with
FNAIT treated by direct foetal immunoglobulin infusion. Foetal treatment was begun
between 17 and 25 weeks and continued until 36 weeks with weekly cordocenteses and
foetal immunoglobulin infusions.RESULTS: While foetal IgG levels increased steadily
during weekly IgG infusions, foetal platelet counts remained unchanged.CONCLUSION:
28
Our retrospective study presents a unique analysis of a historical cohort, contributing to
the ongoing debate about the treatment of choice for foetal alloimmune
thrombocytopenia. Copyright 2010 The Author(s). Vox Sanguinis Copyright 2010
International Society of Blood Transfusion.
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
36. Animal model of fetal and neonatal immune thrombocytopenia: role of neonatal
Fc receptor in the pathogenesis and therapy.
Author(s): Chen P, Li C, Lang S, Zhu G, Reheman A, Spring CM, Freedman J, Ni H
Citation: Blood, November 2010, vol./is. 116/18(3660-8), 0006-4971;1528-0020 (2010 Nov
4)
Publication Date: November 2010
Abstract: Fetal and neonatal immune thrombocytopenia (FNIT) is a severe bleeding
disorder in which maternal antibodies cross the placenta and destroy fetal/neonatal
platelets. It has been demonstrated that the neonatal Fc receptor (FcRn) regulates
immunoglobulin G (IgG) homeostasis and plays an important role in transplacental IgG
transport. However, the role of FcRn in the pathogenesis and therapy of FNIT has not
been studied. Here, we developed an animal model of FNIT using combined beta3
integrin-deficient and FcRn-deficient (beta3(-/-)FcRn(-/-)) mice. We found that beta3(-/)FcRn(-/-) mice are immunoresponsive to beta3(+/+)FcRn(-/-) platelets. The generated
antibodies were beta3 integrin specific and were maintained at levels that efficiently
induced thrombocytopenia in adult beta3(+/+)FcRn(-/-) mice. FNIT was observed when
immunized beta3(-/-)FcRn(+/+) females were bred with beta3(+/+)FcRn(+/+) males, while
no FNIT occurred in beta3(-/-)FcRn(-/-) females bred with beta3(+/+)FcRn(-/-) males,
suggesting that FcRn is indispensable for the induction of FNIT. We further demonstrated
that fetal FcRn was responsible for the transplacental transport of various IgG isotypes.
We found that anti-FcRn antibody and intravenous IgG prevented FNIT, and that
intravenous IgG ameliorated FNIT through both FcRn-dependent and -independent
pathways. Our data suggest that targeting FcRn may be a potential therapy for human
FNIT as well as other maternal pathogenic antibody-mediated diseases.
Source: MEDLINE
Full Text:
Available in fulltext at Highwire Press
37. [Immune thrombocytopenia--pathophysiology and treatment]. [Norwegian]
Immunologisk trombocytopeni--patofysiologi og behandling.
Author(s): Ghanima W, Holme PA, Tjonnfjord GE
Citation: Tidsskrift for Den Norske Laegeforening, November 2010, vol./is. 130/21(21203), 0029-2001;0807-7096 (2010 Nov 4)
Publication Date: November 2010
Abstract: BACKGROUND: Immune thrombocytopenia (ITP) is caused by immune-mediated
platelet destruction and reduced platelet production. The aim of this review article is to
provide an updated overview of pathophysiology and new therapeutic modalities in
ITP.MATERIAL AND METHODS: The article is based on literature identified through a nonsystematic search in PubMed and our own clinical experience.RESULTS: ITP is diagnosed in
patients with platelet count < 100 x 10(9)/l after excluding other causes of
29
thrombocytopenia. Anti-platelet autoantibodies are important in the platelet destruction
mechanism, but other important mechanisms have been identified in recent years.
Patients with very low platelet count < 30 x 10(9)/l are particularly susceptible to bleeding
complications. The goal of treatment so far has been to increase the platelet count to a
level that reduces the risk of serious bleeding. Thrombopoietin receptor agonists are new
therapeutic agents that target the thrombopoietin receptor to increase platelet
production. These drugs are shown to be effective in treatment of ITP.INTERPRETATION:
New knowledge about pathophysiological mechanisms, such as sub-optimal platelet
production in ITP, has led to the development of new therapeutic options which focus on
stimulation of platelet production.
Source: MEDLINE
38. Reconsidering fetal and neonatal alloimmune thrombocytopenia with a focus on
screening and prevention.
Author(s): Skogen B, Killie MK, Kjeldsen-Kragh J, Ahlen MT, Tiller H, Stuge TB, Husebekk A
Citation: Expert Review of Hematology, October 2010, vol./is. 3/5(559-66), 17474094;1747-4094 (2010 Oct)
Publication Date: October 2010
Abstract: Uncertainty regarding the pathophysiology of fetal and neonatal alloimmune
thrombocytopenia (FNAIT) has hampered the decision regarding how to identify, followup and treat the women and children with this potentially serious condition. Since
knowledge of the condition is derived mainly from retrospective studies, understanding of
the natural history of this condition remains incomplete. General screening programs for
FNAIT have still not been introduced, mainly because of a lack of reliable risk factors and
effective treatment. Now, several prospective screening studies involving up to 100,000
pregnant women have been published and the results have changed the understanding of
the pathophysiology of FNAIT and, thereby, the approach toward diagnostics, prevention
and treatment in a more appropriate way.
Source: MEDLINE
39. A retrospective analysis of obstetric patients with idiopathic thrombocytopenic
purpura: a single center study.
Author(s): Fujita A, Sakai R, Matsuura S, Yamamoto W, Ohshima R, Kuwabara H, Okuda M,
Takahashi T, Ishigatsubo Y, Fujisawa S
Citation: International Journal of Hematology, October 2010, vol./is. 92/3(463-7), 09255710;1865-3774 (2010 Oct)
Publication Date: October 2010
Abstract: Idiopathic thrombocytopenic purpura (ITP) commonly affects women of
childbearing age. We studied the clinical characteristics of pregnant women with ITP to
estimate their risks of bleeding. A retrospective chart review was performed for all
obstetric patients with ITP who had delivery at our hospital, from 1 March 2000 to 31
March 2008. Twenty women with ITP delivered 24 children in 23 pregnancies. In all, eight
women were treated with corticosteroid during their pregnancy period, and there was
only one non-responder. There was no correlation between the maternal platelet count
and the amount of blood loss at delivery. Two infants were revealed to have had platelet
counts lower than 30 x 109/L, and were treated with high-dose IV IgG. One of them also
received corticosteroid therapy. There was no relationship between maternal platelet
count at delivery and infant platelet count at birth. Overall, no serious bleeding event was
seen in either of the mothers or infants. For most women with ITP, pregnancy is
uncomplicated, and even those with severe thrombocytopenia during pregnancy have
30
good outcomes when under the strict care of a hematologist and gynecologist.
Source: MEDLINE
40. Bilateral retinal detachments and preeclampsia: thrombotic thrombocytopenic
purpura or syndrome of haemolysis, elevated liver enzymes, low platelets?.
Author(s): Mendez-Figueroa H, Davidson C
Citation: Journal of Maternal-Fetal & Neonatal Medicine, October 2010, vol./is.
23/10(1268-70), 1476-4954;1476-4954 (2010 Oct)
Publication Date: October 2010
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
41. Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia:
systematic review.
Author(s): Kamphuis MM, Paridaans N, Porcelijn L, De Haas M, Van Der Schoot CE, Brand
A, Bonsel GJ, Oepkes D
Citation: BJOG: An International Journal of Obstetrics & Gynaecology, October 2010,
vol./is. 117/11(1335-43), 1470-0328;1471-0528 (2010 Oct)
Publication Date: October 2010
Abstract: BACKGROUND: [en space] Fetal and neonatal alloimmune thrombocytopenia
(FNAIT) is a potentially devastating disease, which may lead to intracranial haemorrhage
(ICH), with neurological damage as a consequence. In the absence of screening, FNAIT is
only diagnosed after bleeding symptoms, with preventive options limited to a next
pregnancy.OBJECTIVES: [en space] To estimate the population incidence of FNAIT and its
consequences to prepare for study design of a screening programme.SEARCH STRATEGY:
[en space] An electronic literature search using MEDLINE, EMBASE and Cochrane
database, and references of retrieved articles. No language restrictions were
applied.SELECTION CRITERIA: [en space] Prospective studies on screening for human
platelet antigen 1a (HPA-1a) alloimmunisation in low-risk pregnant women.DATA
COLLECTION AND ANALYSIS: [en space] Two reviewers independently assessed studies for
inclusion and extracted data. Main outcome data were prevalence of HPA-1a negativity,
HPA-1a immunisation, platelet count at birth and perinatal ICH. We aimed to compare
outcome with and without intervention.MAIN RESULTS: [en space] HPA-1a
alloimmunisation occurred in 294/3028 (9.7%) pregnancies at risk. Severe FNAIT occurred
in 71/227 (31%) immunised pregnancies, with perinatal ICH in 7/71 (10%). True natural
history data were not found because interventions were performed in most screenpositive women.AUTHORS' CONCLUSIONS: [en space] Screening for HPA-1a
alloimmunisation detects about two cases in 1000 pregnancies. The calculated risk for
perinatal ICH of 10% in pregnancies with severe FNAIT is an underestimation because
studies without interventions were lacking. Screening of all pregnancies together with
effective antenatal treatment such as intravenous immunoglobulin may reduce the
mortality and morbidity associated with FNAIT.
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
Available in fulltext at the ULHT Library and Knowledge Services' eJournal collection ; Note:
31
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42. Subdural haematoma in pregnancy-induced idiopathic thrombocytopenia:
Conservative management.
Author(s): Pandey M, Saraswat N, Vajifdar H, Chaudhary L
Citation: Indian Journal of Anaesthesia, September 2010, vol./is. 54/5(470-1), 00195049;0976-2817 (2010 Sep)
Publication Date: September 2010
Abstract: Conservative management of subdural haematoma with antioedema measures
in second gravida with idiopathic thrombocytopenic purpura (ITP) resulted in resolution of
haematoma. We present a case of second gravida with ITP who developed subdural
haematoma following normal vaginal delivery. She was put on mechanical ventilation and
managed conservatively with platelet transfusion, Mannitol 1g/kg, Dexamethasone
1mg/kg and Glycerol 10ml TDS. She regained consciousness and was extubated after 48
hrs. Repeat CT after 10 days showed no mass effect with resolving haematoma which
resolved completely after 15 days. Trial of conservative management is safe in pregnant
patient with ITP who develops subdural haematoma.
Source: MEDLINE
Full Text:
Available in fulltext at National Library of Medicine
43. Successful prevention of thrombotic thrombocytopenic purpura (TTP) relapse
using monthly prophylactic plasma exchanges throughout pregnancy in a patient with
systemic lupus erythematosus and a prior history of refractory TTP and recurrent fetal
loss.
Author(s): Abou-Nassar K, Karsh J, Giulivi A, Allan D
Citation: Transfusion & Apheresis Science, August 2010, vol./is. 43/1(29-31), 14730502;1473-0502 (2010 Aug)
Publication Date: August 2010
Abstract: BACKGROUND: The occurrence of thrombotic thrombocytopenic purpura (TTP)
in the setting systemic lupus erythematosus (SLE) is rare. In women of childbearing age,
TTP is associated with high rates of recurrence in pregnancy. Furthermore, both TTP and
SLE are associated with a significant risk of adverse pregnancy outcomes.CASE
PRESENTATION: We describe the case of a 36 year old female in her first trimester of
pregnancy with a prior history of SLE-associated severe refractory TTP who was treated
with a combination of corticosteroids and prophylactic plasma exchanges (PLEX)
throughout pregnancy to prevent TTP recurrence. She delivered a healthy infant at 33
weeks of gestation after the onset of preterm labor. There was no evidence of TTP
recurrence in the antepartum or postpartum period in this high risk patient.CONCLUSION:
Prophylactic PLEX should be considered as a therapeutic option to prevent recurrent TTP
during pregnancy in high risk patients, including patients with previous SLE-associated
TTP. (c) 2010 Elsevier Ltd. All rights reserved.
Source: MEDLINE
44. [Thrombotic thrombocytopenic purpura in pregnancy. Case report]. [Czech]
Tromboticka trombocytopenicka purpura v tehotenstvi. Kazuistika.
Author(s): Skultety J, Novackova M, Binder T, Hadacova I, Salaj P, Rob L
32
Citation: Ceska Gynekologie, August 2010, vol./is. 75/4(306-8), 1210-7832;1210-7832
(2010 Aug)
Publication Date: August 2010
Abstract: OBJECTIVE: Description of case of patient with rare thrombotic
thrombocytopenic purpura in pregnancy.SUBJECT: Case report.SETTING: Department of
Gynecology and Obstetrics, Charles University and University Hospital Motol,
Prague.CONCLUSION: Thrombotic thrombocytopenic purpura (TTP) is a rare and
substantial disorder characterized with combination of microangiopathic haemolytic
anemia, consumption trombocytopenia and symptoms of organs dysfunction--especially
kidneys and neurological deficiency. It's caused by production of microthrombi affecting
small blood vessels. These palatelets-rich microtrombi are formed due to deficiency of the
enzyme ADAMTS13--metalloprotease which is responsible for cleaving of ultralarge
multimers of von Willebrand factor into smaller units. In our case report we describe
patient with TTP in pregnancy. Therapy with corticosteroids and immunoglobulines was
not effective, improvement of thrombocytopenia appeared after plasmapheresis (total
count 14). The delivery was induced at term without complications. Target examination
confirmed diagnosis of secondary TTP.
Source: MEDLINE
45. Intracranial hemorrhage in alloimmune thrombocytopenia: stratified
management to prevent recurrence in the subsequent affected fetus.
Author(s): Bussel JB, Berkowitz RL, Hung C, Kolb EA, Wissert M, Primiani A, Tsaur FW,
Macfarland JG
Citation: American Journal of Obstetrics & Gynecology, August 2010, vol./is.
203/2(135.e1-14), 0002-9378;1097-6868 (2010 Aug)
Publication Date: August 2010
Abstract: OBJECTIVE: We sought to prevent intracranial hemorrhage (ICH) through
antenatal management of alloimmune thrombocytopenia.STUDY DESIGN: A total of 33
women (37 pregnancies) with alloimmune thrombocytopenia and ICH in a previous child
were stratified according to the timing of the previous child's ICH: extremely high risk (HR)
(n = 8) had ICH <28 weeks, very HR (n = 17) between 28-36 weeks, and HR (n = 12) in the
perinatal period. Treatment was initiated at 12 weeks with intravenous immunoglobulin 1
or 2 g/kg/wk, and if the fetal platelet count by cordocentesis was <30,000/mL despite
treatment, prednisone and/or more intravenous immunoglobulin were added.RESULTS:
Five of 37 fetuses suffered ICHs. Two ICHs had platelet counts >100,000/mL, and 1 was
grade I. The other 2 ICHs were unequivocal treatment failures; both were grade III-IV and
resulted in fetal demise.CONCLUSION: These findings demonstrate the success of
stratified treatment in these HR patients, which tailored interventions according to the
timing of the sibling's ICH. Copyright (c) 2010 Mosby, Inc. All rights reserved.
Source: MEDLINE
46. Postpartum plasma exchange in a woman with suspected thrombotic
thrombocytopenic purpura (TTP) vs. hemolysis, elevated liver enzymes, and low platelet
syndrome (HELLP): a case study.
Author(s): Myers L
Citation: Nephrology Nursing Journal: Journal of the American Nephrology Nurses'
Association, July 2010, vol./is. 37/4(399-402), 1526-744X;1526-744X (2010 Jul-Aug)
Publication Date: July 2010
Abstract: The occurrence of a hypercoagulable state and decreasing concentration of
33
ADAMTS 13 in late pregnancy and during the postpartum period increases the risk for a
woman to develop life-threatening thrombotic thrombocytopenic purpura (TTP). This is
also the time of great risk for the more common obstetric complications of preeclampsia;
eclampsia; and hemolysis, elevated liver functions tests, low platelets (HELLP) syndrome.
These conditions are associated with high maternal and perinatal mortality. Differential
diagnosis may be difficult due to the overlapping of clinical and laboratory findings,
including thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms,
and renal insufficiency, making it difficult or impossible to distinguish them from TTP.
Management of microangiopathic disorders encountered during pregnancy differ;
therefore, an accurate diagnosis is required. Outcomes of TTP without plasma exchange
therapy (TPE) are almost uniformly fatal. Early recognition and management of symptoms
with prompt and aggressive TPE is essential when TTP is suspected.
Source: MEDLINE
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47. A case of pregnancy-induced thrombotic thrombocytopenic purpura with a kidney
allograft recipient.
Author(s): Iwami D, Harada H, Hotta K, Miura M, Seki T, Togashi M, Hirano T
Citation: Clinical Transplantation, July 2010, vol./is. 24 Suppl 22/(66-9), 0902-0063;13990012 (2010 Jul)
Publication Date: July 2010
Abstract: A 32-yr-old female patient, who had been suffering from diffuse crescentic
glomerulonephritis and a consequent end-stage renal disease, successfully underwent
living-related ABO-incompatible kidney transplantation after a desensitization therapy
including anti-CD20 monoclonal antibody. Forty-six months after the transplantation, the
recipient became pregnant. At the 17th gestational week, the patient was admitted for
the management of pregnancy-induced hypertension and aggressive deterioration of
kidney graft function. At the 21st gestational week, the patient lost her kidney graft and
was re-induced into regular hemodialysis. The patient was also suffering from progressive
hemolytic anemia, thrombocytopenia, and neurologic symptoms with decreased activity
of von Willebrand factor-cleaving protease, a disintegrin-like and metalloprotease with
thrombospondin type 1 motifs 13 (ADAMTS13). From these findings and a kidney allograft
biopsy, the patient was diagnosed as thrombotic thrombocytopenic purpura concurrent
with acute T-cell-mediated rejection. The patient immediately underwent plasma
exchange as well as steroid pulse therapy. Despite these treatments, thrombocytopenia
and intrauterine growth retardation progressed. The patient underwent a caesarian
section at the 24th gestational week. Consequently, her platelet count recovered
drastically. However, the patient lost her neonate five d after giving a birth, and the
patient's graft function had never recovered.
Source: MEDLINE
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48. Successful management of recurrent pregnancy-related thrombotic
thrombocytopaenia purpura in a renal transplant recipient.
Author(s): Lam K, Martlew V, Walkinshaw S, Alfirevic Z, Howse M
Citation: Nephrology Dialysis Transplantation, July 2010, vol./is. 25/7(2378-80), 093134
0509;1460-2385 (2010 Jul)
Publication Date: July 2010
Abstract: Thrombotic thrombocytopaenic purpura (TTP) is a rare but potentially
devastating complication of pregnancy. We report the first documented case of a
successful treatment of recurrent TTP complicating pregnancy in a renal transplant
patient.
Source: MEDLINE
Full Text:
Available in fulltext at Highwire Press
49. Predictors for neonatal thrombocytopenia in infants of thrombocytopenic
mothers during pregnancy.
Author(s): Maayan-Metzger A, Leibovitch L, Schushan-Eisen I, Strauss T, Kenet G, Kuint J
Citation: Pediatric Blood & Cancer, July 2010, vol./is. 55/1(145-8), 1545-5009;1545-5017
(2010 Jul 15)
Publication Date: July 2010
Abstract: BACKGROUND: Although maternal thrombocytopenia during pregnancy is
common, its effect on neonatal platelets has not yet been fully evaluated.METHODS: We
retrospectively evaluated the rate of thrombocytopenia among 767 healthy term
neonates (gestational age 37-42 weeks) born to 723 mothers with pregnancy-induced
thrombocytopenia to define risk factors predicting thrombocytopenia in this
group.RESULTS: Thrombocytopenia was diagnosed in 2.2% of the infants. Multivariate
analysis showed that infants with thrombocytopenia were more likely to be male, to be
born at lower gestational age and to have lower birth weight associated with lower
maternal platelets counts. Maternal platelet counts of 100-149 x 10(9)/L, 50-99 x 10(9)/L,
and <50 x 10(9)/L corresponded respectively to 1.7%, 4.3%, and 12.5% of neonatal
thrombocytopenia (P = 0.031).CONCLUSIONS: Routine blood counts are recommended, in
particular for male infants with low birth weight born to mothers with moderate-tosevere thrombocytopenia.
Source: MEDLINE
50. [Clinical analysis of pregnancy complicated with severe thrombocytopenia].
Author(s): Wang DP, Liang MY, Wang SM
Citation: Chung-Hua Fu Chan Ko Tsa Chih [Chinese Journal of Obstetrics & Gynecology],
June 2010, vol./is. 45/6(401-5), 0529-567X;0529-567X (2010 Jun)
Publication Date: June 2010
Abstract: OBJECTIVE: To investigate the etiology and perinatal outcome of pregnancies
complicated with extremely severe thrombocytopenia [at least two times of platelets
count (PLT) < 10 x 10(9)/L during pregnancy].METHODS: Clinical data, including basic
information, etiology, management and outcomes of pregnant women with extremely
severe thrombocytopenia, admitted to Peking University People's Hospital from January
2004 to March 2009, were retrospectively collected. The management of these cases
varied according to different etiology and the symptoms: (1) PLT were maintained > 20 x
10(9)/L and hemoglobulin > 70 g/L in those women without spontaneous bleeding; (2) PLT
transfusion would be required when PLT < 10 x 10(9)/L or bleeding occur and RBC would
be supplied when hematocrit < 25% and hemoglobulin < 70 g/L; (3) Hemoglobulin should
be > 70 g/L and PLT > 30 x 10(9)/L before cesarean section or delivery; (4) Predinisone
and/or intravenous immunoglobulin G (IVIG) would be given in women complicated with
35
idiopathic thrombocytopenic purpura (ITP) when PLT < (20 - 30) x 10(9)/L or bleeding. PLT
would be given if all the above management were failed, or PLT < 10 x 10(9)/L, or
bleeding. Women without bleeding would be closely monitored and delivery would be
planned.RESULTS: (1) Twenty-six cases were identified among 9302 deliveries during the
study period (0.28%), with an average of maternal age of 29. Seventeen were diagnosed
before conception and 9 during pregnancy. Among the 26 women, half received regular
prenatal check in our hospital and the average gestations at diagnosis was 24 weeks and
the other half without regular prenatal visits and the average gestations at diagnosis was
32 weeks. Etiology was identified in 24 out of the 26 women, including 14 (54%) ITP, 5
myelodysplastic syndrome (MDS), 4 chronic aplastic anaemia (CAA) and 1 systemic lupus
erythematosus (SLE). (2) Management: All of the 26 women received blood products.
Among the 14 ITP cases, 6 received predinisone and IVIG and 8 only took predinisone.
Nine of the 26 patients (35%) had pregnant complications, among which 6 (6/9) were
preeclampsia. The overall average gestation at delivery was 36 weeks. Only 2 delivered
vaginally with the average blood loss of 83 ml and 23 cesarean sections were performed
with the average blood loss of 410 ml. (3) Perinatal outcomes: There were 26 perinatal
babies, among which 1 died intrauterine and 25 were born alive (12 preterm infants). The
average birth weight was 2877 g. Neonatal severe thrombocytopenia presented in 2
newborns whose mother complicated with ITP.CONCLUSIONS: The main cause of
extremely severe thrombocytopenia during pregnancy is ITP, managed mainly by
predinisone and IVIG, followed by CAA and MDS, which may require supportive
treatment. Pregnancy complicated with extremely severe thrombocytopenia is not an
indication of termination. Better maternal and fetal outcomes can be achieved through
proper treatment based on the etiology, intensive care in prevention and management of
complications and cesarean section.
Source: MEDLINE
51. The use of angiogenic biomarkers to differentiate non-HELLP related
thrombocytopenia from HELLP syndrome.
Author(s): Young B, Levine RJ, Salahuddin S, Qian C, Lim KH, Karumanchi SA, Rana S
Citation: Journal of Maternal-Fetal & Neonatal Medicine, May 2010, vol./is. 23/5(366-70),
1476-4954;1476-4954 (2010 May)
Publication Date: May 2010
Abstract: OBJECTIVE: Preeclampsia (PE) is diagnosed using clinical criteria and in atypical
cases the diagnosis may be inaccurate as there are no specific tests to confirm or exclude
PE. This study sought to evaluate the utility of angiogenic biomarkers, sFlt1, sEng and PlGF
to distinguish patients with gestational thrombocytopenia and immune thrombocytopenic
purpura (ITP) from patients with thrombocytopenia resulting from the HELLP (hemolysis,
elevated liver enzymes and low platelets) syndrome, a complication of severe
PE.METHODS: Serum was collected and the angiogenic biomarkers of patients with ITP
and gestational thrombocytopenia (N = 9) were compared to patients with HELLP (N = 11)
and PE (N = 11). Circulating levels of these angiogenic biomarkers were also compared by
gestational age to 1564 randomly selected normotensive women from the Calcium for
Preeclampsia Prevention study.RESULTS: Patients with non-HELLP thrombocytopenia had
lower sFlt1 (7.3 +/- 3.8 ng/ml vs. 15.5 +/- 5 ng/ml, P < 0.001), lower sEng (8.7 +/- 3.6 vs. 34
+/- 17, P < 0.001) and higher PlGF (484 +/- 412 vs. 66.3 +/- 44, P = 0.003) than patients
with HELLP syndrome. Angiogenic factor abnormalities in patients with PE were similar to
patients with HELLP syndrome, suggesting a common pathogenesis. Patients with nonHELLP thrombocytopenia had angiogenic profiles similar to normotensive controls,
whereas patients with HELLP syndrome had levels higher than the 90th percentile for sFlt1
and sEng and lower than the 10th percentile for PlGF.CONCLUSIONS: Angiogenic
36
biomarkers may be useful in excluding conditions that mimic PE.
Source: MEDLINE
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52. Prenatal diagnosis of fetal intracranial hemorrhage in pregnancy complicated by
idiopathic thrombocytopenic purpura.
Author(s): Koyama S, Tomimatsu T, Sawada K, Kanagawa T, Isobe A, Taniguchi Y, Wada T,
Kimura T, Arahori H, Kitabatake Y, Wada K
Citation: Prenatal Diagnosis, May 2010, vol./is. 30/5(489-91), 0197-3851;1097-0223 (2010
May)
Publication Date: May 2010
Source: MEDLINE
53. Retrospective comparison of maternal vs. HPA-matched donor platelets for
treatment of fetal alloimmune thrombocytopenia.
Author(s): Giers G, Wenzel F, Fischer J, Stockschlader M, Riethmacher R, Lorenz H,
Tutschek B
Citation: Vox Sanguinis, April 2010, vol./is. 98/3 Pt 2(423-30), 0042-9007;1423-0410 (2010
Apr)
Publication Date: April 2010
Abstract: BACKGROUND AND OBJECTIVES: In fetal alloimmune thrombocytopenia (FAIT),
transplacental maternal antibodies cause destruction of fetal platelets. FAIT is similar to
fetal Rhesus haemolytic disease, but half of the affected fetuses are born to primiparous
women. In 10-20% of cases, prenatal and perinatal intracranial haemorrhages are
reported. Different therapeutic approaches have been described, including maternally
administered high-dose intravenous immunoglobulin (high dose IVIG) without or with
steroids or intrauterine transfusion (IUT) of compatible platelets. For the latter, the use of
plasma-free maternal and donor platelets has been described, but a comparison of these
two sources of platelets has not been reported.MATERIALS AND METHODS: We
retrospectively analyzed the clinical courses of cases with FAIT treated with IUT of either
HPA-matched donor platelets or maternal platelets, done by a single team between 1990
and 1997. In 57 pregnancies, FAIT was treated by repeated IUT with either maternal (15
fetuses) or donor platelets (42 fetuses).RESULTS: There was no procedure-related fetal or
neonatal loss. Platelets from both sources reliably raised the fetal platelet counts. Donor
platelet preparations contained more platelets and yielded higher fetal post-transfusion
platelet counts, but maternal platelets were clinically equally effective.CONCLUSIONS:
Donor and maternal platelet concentrates are effective sources for the treatment of FAIT.
Source: MEDLINE
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54. New low-frequency platelet glycoprotein polymorphisms associated with
neonatal alloimmune thrombocytopenia.
Author(s): Peterson JA, Gitter ML, Kanack A, Curtis B, McFarland J, Bougie D, Aster R
Citation: Transfusion, February 2010, vol./is. 50/2(324-33), 0041-1132;1537-2995 (2010
37
Feb)
Publication Date: February 2010
Abstract: BACKGROUND: Recent reports suggest that maternal immunization against lowfrequency, platelet (PLT)-specific glycoprotein (GP) polymorphisms is a more common
cause of neonatal alloimmune thrombocytopenia (NATP) than previously thought.STUDY
DESIGN AND METHODS: Serologic and molecular studies were performed on PLTs and
DNA from three families in which an infant was born with apparent NATP not attributable
to maternal immunization against known PLT-specific alloantigens.RESULTS: Antibodies
reactive only with paternal PLTs were identified in each mother. In Cases 2 (Kno) and 3
(Nos), but not Case 1 (Sta), antibody recognized paternal GPIIb/IIIa in solid-phase assays.
Unique mutations encoding amino acid substitutions in GPIIb (Case 2) or GPIIIa (Cases 1
and 3) were identified in paternal DNA and in DNA from two of the affected infants.
Antibody from all three cases recognized recombinant GPIIIa (Case 1 [Sta] and Case 3
[Nos]) and GPIIb (Case 2, Kno) mutated to contain the polymorphisms identified in the
respective fathers. None of 100 unselected normal subjects possessed the paternal
mutations. Enzyme-linked immunosorbent assay and flow cytometric studies suggested
that failure of maternal serum from Case 1 (Sta) to react with paternal GPIIIa in solidphase assays resulted from use of a monoclonal antibody AP2, for antigen immobilization
that competed with the maternal antibody for binding to the Sta epitope.CONCLUSION:
NATP in the three cases was caused by maternal immunization against previously
unreported, low-frequency GP polymorphisms. Maternal immunization against lowfrequency PLT-specific alloantigens should be considered in cases of apparent NATP not
resolved by conventional serologic and molecular testing.
Source: MEDLINE
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if required.
55. Cerebellar loss and brain-stem atrophy associated with neonatal alloimmune
thrombocytopenia in a discordant twin.
Author(s): Mohila CA, Kubicka ZJ, Ornvold KT, Harris BT
Citation: Pediatric & Developmental Pathology, January 2010, vol./is. 13/1(55-62), 10935266;1093-5266 (2010 Jan-Feb)
Publication Date: January 2010
Abstract: Neonatal alloimmune thrombocytopenia (NAIT) is due to an immune-mediated
maternal-fetal platelet antigen incompatibility. Central nervous system abnormalities
have been reported in infants with NAIT and include intracranial hemorrhage,
ventriculomegaly, porencephalic cysts, neuronal migrational disorders, and, rarely,
cerebellar lesions. We present the clinical and neuropathological findings from a case of a
3-day-old diamniotic/dichorionic female twin with known bilateral ventriculomegaly born
prematurely at 33-1/7 weeks in gestational age. The pregnancy was further complicated
by discordant intrauterine growth, intraventricular hemorrhage in the co-twin, and NAIT.
At birth, the infant was noted to have diffuse body ecchymoses and petechiae and
arthrogryposis. She subsequently developed multisystem organ failure and disseminated
intravascular coagulopathy and died on the 3rd day of life. Neuropathological findings at
autopsy included a posterior fossa cyst with no gross anatomic evidence of a cerebellum,
atrophic pons and medulla with prominent pyramidal tracts and absent olivary nuclei,
thinned corpus callosum, and symmetrical dilation of bilateral lateral ventricles.
38
Microscopic examination confirmed the gross findings and revealed no histological
evidence of cerebellar tissue, absence of superior and inferior cerebellar peduncles, and
acute and chronic germinal matrix hemorrhages. Immunohistochemical studies revealed a
focus of reactive gliosis at the base of the posterior fossa cyst with no evidence of
cerebellar Purkinje or granule cells. To our knowledge, this is the 1st report with wellcharacterized neuropathological examination detailing complete cerebellar loss and brainstem atrophy in a neonate with NAIT.
Source: MEDLINE
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56. Successful pregnancy in a case of congenital thrombotic thrombocytopenic
purpura.
Author(s): Meti S, Paneesha S, Patni S
Citation: Journal of Obstetrics & Gynaecology, 2010, vol./is. 30/5(519-21), 01443615;1364-6893 (2010)
Publication Date: 2010
Source: MEDLINE
Full Text:
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57. Perinatal management of immune thrombocytopenic purpura--a case report and
review of literature.
Author(s): Gupta RS, Rajaram S, Bharadwaj P, Goel N, Singh KC, Bisht S
Citation: Journal of the Indian Medical Association, January 2010, vol./is. 108/1(42, 47-8),
0019-5847;0019-5847 (2010 Jan)
Publication Date: January 2010
Abstract: Immune thrombocytopenic purpura is principally a disease of young women.
Therefore it may often be associated with pregnancy. It is commonly complicated by
abortion, intra-uterine growth retardation and neonatal intracranial haemorrhage so that
perinatal mortality may be as high as 20%. Hence perinatal management of immune
thrombocytopenic purpura should include maintenance of maternal platelet count and
regular monitoring of foetal growth along with prediction and prevention of foetal passive
immune thrombocytopenia. Determination of foetal platelet count in certain situations
may help in concomitant selection of delivery mode. The following case report emphasises
the importance of diagnosing this condition at peripheral healthcare level so that
perinatal outcome can be markedly improved.
Source: MEDLINE
58. Treatment of idiopathic thrombocytopenic purpura in pregnancy with pulsed dose
of dexamethasone.
Author(s): Grgic O, Ivanisevic M, Delmis J
Citation: Journal of Obstetrics & Gynaecology, 2010, vol./is. 30/8(864), 0144-3615;13646893 (2010)
Publication Date: 2010
Source: MEDLINE
39
Full Text:
Available in fulltext at EBSCO Host
59. Pregnancy-associated thrombotic thrombocytopenic purpura with anticentromere antibody-positive Raynaud's syndrome.
Author(s): Watanabe R, Shirai T, Tajima Y, Ohguchi H, Onishi Y, Fujii H, Takasawa N, Ishii T,
Harigae H
Citation: Internal Medicine, 2010, vol./is. 49/12(1229-32), 0918-2918;1349-7235 (2010)
Publication Date: 2010
Abstract: Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and
hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome
display common symptoms that include microangiopathic hemolytic anemia,
thrombocytopenia, and renal failure. Therefore, it is important to distinguish between
them because their treatments vary: however, the differential diagnosis is sometimes
difficult. We report a 32-year-old woman who was referred to our department for further
examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly
elevated serum creatinine level with anti-centromere antibody-positive Raynaud's
syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered
in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease
with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to
the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose
prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic
anemia and thrombocytopenia are observed in perinatal women or patients with signs of
systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are
essential for diagnosis and therapeutic choice.
Source: MEDLINE
60. Anticoagulation with argatroban in a parturient with heparin-induced
thrombocytopenia.
Author(s): Ekbatani A, Asaro LR, Malinow AM
Citation: International Journal of Obstetric Anesthesia, January 2010, vol./is. 19/1(82-7),
0959-289X;1532-3374 (2010 Jan)
Publication Date: January 2010
Abstract: Unfractionated heparin and low-molecular-weight heparin are currently the
anticoagulants of choice for the prevention of recurrent thromboembolic disease during
pregnancy. However, heparin-induced thrombocytopenia contraindicates the use of
unfractionated heparin and low-molecular-weight heparin. We describe a patient who
was admitted to our hospital with deep vein thrombosis at 18 weeks of gestation and who
developed heparin-induced thrombocytopenia during her antenatal care. Therapeutic
anticoagulation was initially achieved with argatroban, then changed to fondaparinux.
During early labor, fondaparinux was discontinued and intravenous argatroban was
substituted. Argatroban was discontinued during transition to active labor. After return of
a normal partial thromboplastin time, combined spinal-epidural analgesia was induced for
routine completion of labor and vaginal delivery. We discuss the decisions made in the
maintenance of this patient's anticoagulation during the peripartum period as well as
timing of her neuraxial labor analgesia. Copyright 2009 Elsevier Ltd. All rights reserved.
Source: MEDLINE
61. The risk of spinal haematoma following neuraxial anaesthesia or lumbar puncture
40
in thrombocytopenic individuals.
Author(s): van Veen JJ, Nokes TJ, Makris M
Citation: British Journal of Haematology, January 2010, vol./is. 148/1(15-25), 00071048;1365-2141 (2010 Jan)
Publication Date: January 2010
Abstract: Neuraxial anaesthesia is increasingly performed in thrombocytopenic patients at
the time of delivery of pregnancy. There is a lack of data regarding the optimum platelet
count at which spinal procedures can be safely performed. Reports are often confounded
by the presence of other risk factors for spinal haematomata, such as anticoagulants,
antiplatelet agents and other acquired or congenital coagulopathies/platelet function
defects or rapidly falling platelet counts. In the absence of these additional risk factors, a
platelet count of 80 x 10(9)/l is a 'safe' count for placing an epidural or spinal anaesthetic
and 40 x 10(9)/l is a 'safe' count for lumbar puncture. It is likely that lower platelet counts
may also be safe but there is insufficient published evidence to make recommendations
for lower levels at this stage. For patients with platelet counts of 50-80 x 10(9)/l requiring
epidural or spinal anaesthesia and patients with a platelet count 20-40 x 10(9)/l requiring
a lumbar puncture, an individual decision based on assessment of risks and benefits
should be made.
Source: MEDLINE
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62. Neonatal outcomes of pregnancy complicated by idiopathic thrombocytopenic
purpura.
Author(s): Ozkan H, Cetinkaya M, Koksal N, Ali R, Gunes AM, Baytan B, Ozkalemkas F,
Ozkocaman V, Ozcelik T, Gunay U, Tunali A, Kimya Y, Cengiz C
Citation: Journal of Perinatology, January 2010, vol./is. 30/1(38-44), 0743-8346;1476-5543
(2010 Jan)
Publication Date: January 2010
Abstract: OBJECTIVE: The aim of this study was to determine the factors associated with
the prognosis of newborns born to mothers with idiopathic thrombocytopenic purpura
(ITP), and to compare the infants with/without thrombocytopenia in terms of maternal
and neonatal characteristics.STUDY DESIGN: We reviewed the charts of 29 parturients
with ITP and their newborns who were born between January 1998 and December
2008.RESULT: A total of 16 (55%) gravidas had been diagnosed with ITP before pregnancy
and 13 (45%) were diagnosed during pregnancy. Thrombocytopenia was observed in 21
gravidas. In total, 17 (58%) gravidas received treatment to increase the platelet count. The
majority of deliveries (72.5%) were vaginal. The infant platelet counts at birth ranged from
20 to 336 x 10(9) per liter. None of the neonates had complications attributable to the
mode of delivery. Normal platelet counts were determined in 15 newborns, whereas 14
infants had thrombocytopenia at birth. Three (10.3%) neonates had mild, four neonates
(13.7%) had moderate and seven neonates (24.1%) had severe thrombocytopenia. The
age of the mothers having infants with thrombocytopenia was significantly higher (30+/5.3 vs 25.3+/-3.8 years), most of the infants (10/14 (71%)) were males
(P<0.05).CONCLUSION: Pregnancy complicated with ITP generally has a good outcome.
Although ITP in pregnancy carries a low risk, careful observation is required for the
newborn of gravidas with ITP even when the infant has no bleeding complications at
delivery, and infants may require treatment for thrombocytopenia.
41
Source: MEDLINE
63. International consensus report on the investigation and management of primary
immune thrombocytopenia.
Author(s): Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, Chong
BH, Cines DB, Gernsheimer TB, Godeau B, Grainger J, Greer I, Hunt BJ, Imbach PA, Lyons G,
McMillan R, Rodeghiero F, Sanz MA, Tarantino M, Watson S, Young J, Kuter DJ
Citation: Blood, January 2010, vol./is. 115/2(168-86), 0006-4971;1528-0020 (2010 Jan 14)
Publication Date: January 2010
Abstract: Previously published guidelines for the diagnosis and management of primary
immune thrombocytopenia (ITP) require updating largely due to the introduction of new
classes of therapeutic agents, and a greater understanding of the disease
pathophysiology. However, treatment-related decisions still remain principally dependent
on clinical expertise or patient preference rather than high-quality clinical trial evidence.
This consensus document aims to report on new data and provide consensus-based
recommendations relating to diagnosis and treatment of ITP in adults, in children, and
during pregnancy. The inclusion of summary tables within this document, supported by
information tables in the online appendices, is intended to aid in clinical decision making.
Source: MEDLINE
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64. A Case of HELLP Syndrome in a Patient with Immune Thrombocytopenic Purpura.
Author(s): Ben S, Rodriguez F, Severo C, Debat N
Citation: Obstetrics & Gynecology International, 2010, vol./is. 2010/, 1687-9597 (2010)
Publication Date: 2010
Abstract: We will describe the clinical case of a pregnant patient with chronic Immune
Thrombocytopenic Purpura who develops preeclampsia syndrome with HELLP syndrome.
These concomitant and independent conditions become complex, resulting in
thrombocytopenia which creates diagnostic, prognostic and therapeutic inconveniences.
Source: MEDLINE
Full Text:
Available in fulltext at National Library of Medicine
65. Thrombocytopenia in pregnancy.
Author(s): McCrae KR
Citation: Hematology, 2010, vol./is. 2010/(397-402), 1520-4383;1520-4383 (2010)
Publication Date: 2010
Abstract: Thrombocytopenia occurs commonly during pregnancy, and may result from
diverse etiologies. Awareness of these many causes facilitates proper diagnosis and
management of thrombocytopenia in the pregnant setting. Some causes of
thrombocytopenia are unique to pregnancy and may not be familiar to hematologists. In
the review, we will discuss the differential diagnosis of thrombocytopenia in pregnancy,
and the pathogenesis of selected thrombocytopenic disorders. Considerations for optimal
management of the pregnant patient with thrombocytopenia will also be described.
42
Source: MEDLINE
Full Text:
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66. Immune thrombocytopenia in pregnancy.
Author(s): Stavrou E, McCrae KR
Citation: Hematology - Oncology Clinics of North America, December 2009, vol./is.
23/6(1299-316), 0889-8588;1558-1977 (2009 Dec)
Publication Date: December 2009
Abstract: Management of immune thrombocytopenia in pregnancy can be a complex and
challenging task and may be complicated by fetal-neonatal thrombocytopenia. Although
fetal intracranial hemorrhage is a rare complication of immune thrombocytopenia in
pregnancy, invasive studies designed to determine the fetal platelet count before delivery
are associated with greater risk than that of fetal intracranial hemorrhage and are
discouraged. Moreover, the risk of neonatal bleeding complications does not correlate
with the mode of delivery, and cesarean section should be reserved only for obstetric
indications.
Source: MEDLINE
67. Prenatal treatment of fetomaternal thrombocytopenia.
Author(s): Vatopoulou T, Sorinola O
Citation: British Journal of Hospital Medicine, November 2009, vol./is. 70/11(660-1), 17508460;1750-8460 (2009 Nov)
Publication Date: November 2009
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
Available in print at Lincoln County Hospital Professional Library
68. Perinatal outcomes and complications of pregnancy in women with immune
thrombocytopenic purpura.
Author(s): Belkin A, Levy A, Sheiner E
Citation: Journal of Maternal-Fetal & Neonatal Medicine, November 2009, vol./is.
22/11(1081-5), 1476-4954;1476-4954 (2009 Nov)
Publication Date: November 2009
Abstract: OBJECTIVE: To investigate pregnancy and perinatal outcomes in women with
immune thrombocytopenic purpura (ITP).METHODS: A retrospective study comparing all
singleton pregnancies of women with and without ITP was conducted. Deliveries occurred
between the years 1988 and 2007. Multiple logistic regression models were performed to
control for confounders.RESULTS: During the study period, 186,602 deliveries were
recorded, out of which 104 (0.06%) occurred in patients with ITP. In a multivariable
analysis, we found the following conditions to be significantly and independently
associated with ITP: hypertensive disorders, diabetes mellitus, and preterm delivery (<34
weeks gestation). Patients with ITP had significantly higher rates of preterm delivery (<34
weeks gestation; 6.7%vs. 2.2%; p < 0.001) and perinatal mortality (4.8%vs. 1.3%; p =
43
0.011) when compared with patients without ITP. Two multivariable logistic regression
models were constructed with perinatal mortality and preterm delivery (<34 weeks
gestation) as the outcome variables to control for possible confounders such as congenital
malformations, hypertension, diabetes mellitus, and maternal age. In these models, ITP
was found to be an independent risk factor for perinatal mortality (OR = 3.77; 95% CI 1.3210.78, p = 0.013), as well as for preterm delivery before 34 weeks gestation (OR = 3.01;
95% CI 1.39-6.52, p = 0.005).CONCLUSION: ITP is significantly and independently
associated with preterm delivery before 34 weeks gestation and with perinatal mortality.
Source: MEDLINE
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69. Successful vaginal delivery in a patient with extreme thrombotic
thrombocytopenic purpura at term.
Author(s): Davies M, Maiti S, Bolton-Maggs PH, Byrd L
Citation: Journal of Obstetrics & Gynaecology, November 2009, vol./is. 29/8(765-6), 01443615;1364-6893 (2009 Nov)
Publication Date: November 2009
Source: MEDLINE
Full Text:
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70. IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired
thrombotic thrombocytopenic purpura.
Author(s): Ferrari S, Mudde GC, Rieger M, Veyradier A, Kremer Hovinga JA, Scheiflinger F
Citation: Journal of Thrombosis & Haemostasis, October 2009, vol./is. 7/10(1703-10),
1538-7836;1538-7836 (2009 Oct)
Publication Date: October 2009
Abstract: BACKGROUND: ADAMTS13-neutralizing IgG autoantibodies are the major cause
of acquired thrombotic thrombocytopenic purpura (TTP).OBJECTIVE: To analyze the IgG
subclass distribution of anti-ADAMTS13 antibodies and a potential relationship between
subclass distribution and disease prognosis.METHODOLOGY: An enzyme-linked
immunosorbent assay-based method was used to quantify the relative amounts of IgG
subclasses of anti-ADAMTS13 antibodies in acquired TTP plasma.RESULTS: IgG(4) (52/58,
90%) was the most prevalent IgG subclass in patients with acquired TTP, followed by
IgG(1) (52%), IgG(2) (50%), and IgG(3) (33%). IgG(4) was found either alone (17/52) or with
other IgG subclasses (35/52). IgG(4) was not detected in 10% of the patients. There was an
inverse correlation between the frequency and abundance of IgG(4) and IgG(1) antibodies
(P < 0.01). Patients with high IgG(4) levels and undetectable IgG(1) are more prone to
relapse than patients with low IgG(4) levels and detectable IgG(1).CONCLUSIONS: All IgG
subclasses of anti-ADAMTS13 antibodies were detected in patients with acquired TTP,
with IgG(4), followed by IgG(1), antibodies dominating the anti-ADAMTS13 immune
response. Levels of IgG(4) could be useful for the identification of patients at risk of
disease recurrence.
Source: MEDLINE
Full Text:
44
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71. Limitations of ADAMTS-13 activity level in diagnosing thrombotic
thrombocytopenic purpura in pregnancy.
Author(s): Ehsanipoor RM, Rajan P, Holcombe RF, Wing DA
Citation: Clinical & Applied Thrombosis/Hemostasis, October 2009, vol./is. 15/5(585-7),
1076-0296;1938-2723 (2009 Oct)
Publication Date: October 2009
Abstract: In pregnancy, it may be difficult to differentiate the syndrome of hemolysis,
elevated liver enzymes, and low platelets from thrombotic thrombocytopenia purpura.
Severely depressed (<5%) or absence of a disintegrin and metalloproteinase with
thrombospondin motifs-13 activity levels are associated with thrombotic
thrombocytopenia purpura and mildly decreased levels are associated with other disease
processes, including pre-eclampsia and hemolysis, elevated liver enzymes, and low
platelets syndrome. We present a case of a patient that presented at 20 weeks gestation
with elevated liver enzymes and thrombocytopenia. The diagnosis was unclear at the time
of presentation. She underwent induction of labor, and during the postpartum course, she
was eventually diagnosed with thrombotic thrombocytopenia purpura; however, her
activity level of a disintegrin and metalloproteinase with thrombospondin motifs-13 was
only moderately depressed at 15% (normal pregnancy value 41%-105%).
Source: MEDLINE
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72. [Effect of general anesthesia used in cesarean section on maternal-neonatal
outcome of pregnancy complicated with severe thrombocytopenia].
Author(s): Wei J, Liu GL, Liang MY, Wang SM
Citation: Chung-Hua Fu Chan Ko Tsa Chih [Chinese Journal of Obstetrics & Gynecology],
September 2009, vol./is. 44/9(665-8), 0529-567X;0529-567X (2009 Sep)
Publication Date: September 2009
Abstract: OBJECTIVE: To investigate the effect of general anesthesia on pregnancy women
with thrombocytopenia and neonate during cesarean section (CS).METHODS: Sixty-five
singleton pregnant women with low platelet count (< 50 x 10(9)/L) and gestation>35
weeks were allocated into general anesthesia group (35 cases) and local anesthesia group
(30 cases) randomly. The time from skin incision to fetal delivery, the oxyhemoglobin
saturation (SO2) before and after anesthesia, the blood loss during operation, Apgar
scores at 1 min, birth weight,umbilical cord blood gas analysis were recorded.RESULTS:
The mean time from anesthesia induction to fetal delivery was (9.7 +/- 3.5) minutes in
general anesthesia group. The time from skin incision to fetal delivery in general
anesthesia group [(7.7 +/- 2.5) minutes] was shorter than that in local anesthesia group
[(12.5 +/- 3.0) minutes, P < 0.01], while the operation time had no significant differences.
There were no significant difference for the value of SO2 before and after general
anesthesia or local anesthesia (P > 0.05). There was no significant difference for the blood
loss [(471 +/- 245) ml vs. (452 +/- 213) ml, P > 0.05], Apgar scores at 1 minute, birth weight
and umbilical cord blood gas analysis between the two groups (P > 0.05). There had two
infants with blue asphyxia in local anesthesia group while no infant with asphyxia in
general anesthesia group.CONCLUSION: General anesthesia is safe to pregnant women
with thrombocytopenia during CS.
45
Source: MEDLINE
73. Successful use of danaparoid in two pregnant women with heart valve prosthesis
and heparin-induced thrombocytopenia Type II (HIT).
Author(s): Gerhardt A, Scharf RE, Zotz RB
Citation: Clinical & Applied Thrombosis/Hemostasis, July 2009, vol./is. 15/4(461-4), 10760296;1076-0296 (2009 Jul-Aug)
Publication Date: July 2009
Abstract: Anticoagulant therapy with heparin for the prevention of thromboembolism in
pregnant women with prosthetic heart valves is associated with an increased risk to the
mother and/or the fetus. A life-threatening complication of the therapy with heparin is
heparin-induced thrombocytopenia type II (HIT). danaparoid has not yet been reported to
be safe and effective for this indication. This study reports on a 26-year-old woman with
tricuspidal valve prosthesis and a 37-year-old woman with a St. Jude Medical mitral valve
prosthesis who were anticoagulated with danaparoid during pregnancy because of HIT.
Anti-Xa levels were between 0.6 and 1.2 IU/mL during pregnancy with target levels of 1.0
IU/mL. Cesarean section was performed at anti-Xa levels of 0.3 and 0.7 IU/mL. One
woman developed a placental hematoma at the 32nd week of gestation, which did not
increase over the following week. Both patients delivered healthy boys. Heparin-induced
thrombocytopenia in pregnant women with prosthetic heart valve can be successfully
managed with danaparoid.
Source: MEDLINE
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74. Diagnosis and management of the fetus and neonate with alloimmune
thrombocytopenia.
Author(s): Bussel J
Citation: Journal of Thrombosis & Haemostasis, July 2009, vol./is. 7 Suppl 1/(253-7), 15387836;1538-7836 (2009 Jul)
Publication Date: July 2009
Abstract: Fetal and neonatal alloimmune thrombocytopenia (AIT) is the commonest cause
of severe thrombocytopenia in neonates, and of intracranial hemorrhage (ICH) in term
neonates [1] (J Trop Pediatr, 1999; 45: 237). If a newborn is affected with AIT, the next
child will likely be more severely affected, and therefore fetal thrombocytopenia will
begin early in gestation [2, 3] (Arch Neurol, 1984; 41: 30; N Engl J Med 1997; 337: 22). This
creates a risk of in utero ICH even if there was not one in the previous pregnancy. There
are new developments in AIT in regard to diagnosis, treatment, and screening which will
be the focus of this review.
Source: MEDLINE
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75. Idiopathic thrombocytopaenic purpura in pregnancy presenting with lifethreatening epistaxis.
Author(s): Bukar M, Audu BM, Bako BG, Garandawa HI, Kagu MB
Citation: Journal of Obstetrics & Gynaecology, July 2009, vol./is. 29/5(439-40), 014446
3615;1364-6893 (2009 Jul)
Publication Date: July 2009
Source: MEDLINE
Full Text:
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76. Neonatal alloimmune thrombocytopenia and neutropenia associated with
maternal human leukocyte antigen antibodies.
Author(s): Gramatges MM, Fani P, Nadeau K, Pereira S, Jeng MR
Citation: Pediatric Blood & Cancer, July 2009, vol./is. 53/1(97-9), 1545-5009;1545-5017
(2009 Jul)
Publication Date: July 2009
Abstract: Neonatal thrombocytopenia or neutropenia may result from passive transfusion
of maternally derived antibodies. Antibodies against platelet antigens are commonly
associated with neonatal alloimmune thrombocytopenia (NAIT), and anti-neutrophil
antibodies are frequently identified in alloimmune neonatal neutropenia (ANN).
Combined alloimmune cytopenias in the newborn are rarely reported; even fewer reports
document human leukocyte antigen (HLA) antibodies as a potential cause of neonatal
thrombocytopenia or neutropenia. We describe neutropenia and thrombocytopenia in a
newborn associated with markedly elevated maternal HLA antibodies in the absence of
anti-neutrophil or anti-platelet antibodies to highlight consideration of HLA antibodies in
the pathogenesis of ANN and NAIT. Copyright 2009 Wiley-Liss, Inc.
Source: MEDLINE
77. The relationship of anti-HPA-1a amount to severity of neonatal alloimmune
thrombocytopenia - Where does it stand?.
Author(s): Bessos H, Killie MK, Seghatchian J, Skogen B, Urbaniak SJ
Citation: Transfusion & Apheresis Science, April 2009, vol./is. 40/2(75-8), 1473-0502;14730502 (2009 Apr)
Publication Date: April 2009
Abstract: The issue of whether or not antibody quantity during pregnancy is related to
severity of neonatal alloimmune thrombocytopenia remains unresolved. In this article we
cite studies in support of both sides of the argument and highlight some of the reasons
that may lie behind the observed differences amongst those studies. It may well be that
some of the reasons for the discrepant results could be due to the type of study carried
out (eg retrospective versus prospective), the sample size, the timing of antibody
sampling, and possibly the type or protocol of assay used. Another major reason is the
absence, until recently, of an international anti-HPA-1a standard.
Source: MEDLINE
78. The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic
uremic syndrome in the obstetric triage and emergency department: lessons from 4
tertiary hospitals.
Author(s): Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, How H, Sibai B
Citation: American Journal of Obstetrics & Gynecology, April 2009, vol./is. 200/4(381.e16), 0002-9378;1097-6868 (2009 Apr)
47
Publication Date: April 2009
Abstract: OBJECTIVE: We report a series of occurrences of thrombotic thrombocytopenic
purpura (TTP)/hemolytic uremic syndrome (HUS) in pregnancy that emphasizes early
diagnosis.STUDY DESIGN: Fourteen pregnancies with TTP (n = 12) or HUS (n = 2) were
studied. Analysis focused on clinical and laboratory findings on examination, initial
diagnosis, and treatment.RESULTS: There were 14 pregnancies in 12 patients; 2 cases of
TTP were diagnosed as recurrent. Five women were admitted to the emergency
department (ED), and 7 patients were admitted to an obstetrics triage. Patients who were
evaluated by an obstetrician were treated initially for hemolysis, elevated liver enzymes
and low platelets syndrome/preeclampsia, whereas patients who were seen in the ED had
a diagnosis that is commonplace in the ED (panic attack, domestic violence,
gastroenteritis). Latency from the onset of symptoms to diagnosis ranged from 1-7 days.
Plasmapheresis treatments in early gestation resulted in favorable maternal-neonatal
outcome. Maternal and perinatal mortality rates were 25% each.CONCLUSION: TTP/HUS is
a challenging diagnosis in obstetric triage and ED areas. We propose a management
scheme that suggests how to triage patients for early diagnosis in pregnancy.
Source: MEDLINE
79. [Anesthetic management in a pregnant woman suffering from idiopathic
thrombocytopenic purpura]. [Spanish] Manejo anestesico en gestante afecta de purpura
trombocitopenica idiopatica.
Author(s): Raynard Ortiz M, Jamart V, Cambray C, Borras R, Mailan J
Citation: Revista Espanola de Anestesiologia y Reanimacion, March 2009, vol./is.
56/3(185-8), 0034-9356;0034-9356 (2009 Mar)
Publication Date: March 2009
Abstract: Idiopathic thrombocytopenic purpura is an autoimmune disorder characterized
by a low platelet count. Onset usually occurs during adolescence with episodes of
cutaneous and mucosal bleeding. Thrombocytopenia during pregnancy is associated with
many diseases, of which idiopathic thrombocytopenic purpura is the most common in the
first trimester. The need for treatment will depend on the platelet count and whether
there is bleeding. At the end of pregnancy, however, whether delivery is vaginal or by
cesarean, more aggressive therapeutic measures are required. Anesthetic management in
this type of patient will be determined by coagulation status and platelet count, and local
or regional anesthesia may be contraindicated. We report the case of a pregnant woman
with idiopathic thrombocytopenic purpura who was admitted to the emergency
department of our hospital with suspected preeclampsia.
Source: MEDLINE
80. Regional anesthesia and non-preeclamptic thrombocytopenia: time to re-think
the safe platelet count.
Author(s): Tanaka M, Balki M, McLeod A, Carvalho JC
Citation: Revista Brasileira de Anestesiologia, March 2009, vol./is. 59/2(142-53), 00347094;1806-907X (2009 Mar-Apr)
Publication Date: March 2009
Abstract: BACKGROUND AND OBJECTIVES: Although regional anesthesia is widely used for
pain control in obstetrics, it may not be appropriate for patients with thrombocytopenia
due to the risk of neuraxial hematoma. There is no strong evidence to suggest the
minimum platelet count that is necessary to ensure the safe practice of regional
anesthesia. The purpose of this study was to review the safety of regional anesthesia in
48
non-preeclamptic thrombocytopenic parturients at our institution over a 5-year
period.METHODS: A retrospective chart review was performed in all the non-preeclamptic
obstetric patients who delivered at our facility between April 2001 and March 2006, and
had platelet counts < 100 x 10(9).L(-1) on the day of anesthesia. The etiology of the
thrombocytopenia, type of anesthesia, mode of delivery and major anesthetic
complications were noted.RESULTS: Seventy-five patients were identified, 47 of whom
(62.6%) had received regional anesthesia. The etiology of their thrombocytopenia was
immune thrombocytopenic purpura in 49 patients, gestational thrombocytopenia in 20
and other causes in 6 patients. Regional anesthesia was administered in 91.9% of the
patients with platelet counts of 80 to 99 x 10(9).L(-1) and in 48.1% of the patients with
platelet counts of 50 to 79 x 10(9).L(-1). None of the 11 patients with platelet counts
below 50 x 10(9).L(-1) received regional anesthesia. There were no neurological
complications.CONCLUSIONS: In our series, regional anesthesia was safely administered in
pregnant patients with platelet counts between 50-79 x 10(9).L(-1). Our results are in
keeping with other series in the literature. We suggest that in non-preeclamptic patients
with stable platelet counts and no history or clinical signs of bleeding, the lower limit of
platelet count for regional anesthesia should be 50 x 10(9).L(-1).
Source: MEDLINE
81. Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in
Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients.
Author(s): Fujimura Y, Matsumoto M, Kokame K, Isonishi A, Soejima K, Akiyama N,
Tomiyama J, Natori K, Kuranishi Y, Imamura Y, Inoue N, Higasa S, Seike M, Kozuka T, Hara
M, Wada H, Murata M, Ikeda Y, Miyata T, George JN
Citation: British Journal of Haematology, March 2009, vol./is. 144/5(742-54), 00071048;1365-2141 (2009 Mar)
Publication Date: March 2009
Abstract: Upshaw-Schulman syndrome (USS) is a congenital thrombotic thrombocytopenic
purpura (TTP) due to mutations in the gene that encodes for ADAMTS13 (ADAMTS13), but
its clinical signs may be mild or absent during childhood. We have identified 37 patients
with USS (24 females, 13 males) belonging to 32 families. The nine women from six
families who were diagnosed during their first pregnancy are the focus of this report. Six
of the nine women had episodes of thrombocytopenia during childhood misdiagnosed as
idiopathic thrombocytopenic purpura. Thrombocytopenia occurred during the secondthird trimesters in each of their 15 pregnancies, with 16 babies (one twin pregnancy),
often followed by TTP. Of 15 pregnancies, eight babies were stillborn or died soon after
birth, and the remaining seven were all premature except one, who was born naturally
following plasma infusions to the mother that had started at 8 weeks' gestation. All nine
USS women had severely deficient ADAMTS13 activity. ADAMTS13 analyses demonstrated
that eight women were compound heterozygotes of Y304C/G525D (2 siblings),
R125VfsX6/Q1302X (2 siblings), R193W/R349C (2 siblings), I178T/Q929X, and
R193W/A606P; one woman was homozygous for R193W. Only the R193W mutation has
been previously reported. These observations emphasize the importance of measuring
ADAMTS13 activity in the evaluation of thrombocytopenia during childhood and
pregnancy.
Source: MEDLINE
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82. Thrombotic thrombocytopenic purpura in the first trimester and successful
49
pregnancy.
Author(s): Trisolini SM, Capria S, Gozzer M, Pupella S, Foa R, Mazzucconi MG, Meloni G
Citation: Annals of Hematology, March 2009, vol./is. 88/3(287-9), 0939-5555;1432-0584
(2009 Mar)
Publication Date: March 2009
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
83. Current approaches to the evaluation and management of the fetus and neonate
with immune thrombocytopenia.
Author(s): Bussel JB, Sola-Visner M
Citation: Seminars in Perinatology, February 2009, vol./is. 33/1(35-42), 0146-0005;1558075X (2009 Feb)
Publication Date: February 2009
Abstract: Fetal and neonatal alloimmune thrombocytopenia is not a well-known disease,
except among specialists in maternal-fetal medicine, neonatologists, and certain
pediatricians (ie, hematologists). However, this is by far the most common cause of early
severe thrombocytopenia in neonates and of intracranial hemorrhage in term neonates.
In addition, if a newborn is affected with alloimmune thrombocytopenia, the next child in
the family will likely be more severely affected. Thus, the accurate diagnosis and
appropriate management of this disorder are of extreme importance in perinatal
medicine and will constitute the focus of this review.
Source: MEDLINE
84. Pregnancy-associated thrombotic thrombocytopenic purpura.
Author(s): Gerth J, Schleussner E, Kentouche K, Busch M, Seifert M, Wolf G
Citation: Thrombosis & Haemostasis, February 2009, vol./is. 101/2(248-51), 03406245;0340-6245 (2009 Feb)
Publication Date: February 2009
Abstract: Thrombocytopenia during pregnancy is a common diagnostic and management
problem. Several differential diagnosis must be considered including manifestations of
thrombotic thrombocytopenic purpura (TTP). We report here on a case of a 21-year-old
pregnant woman who presented initially severe thrombocytopenia (8 Gpt/l) in the
20(th)+1 week of gestation. The patient had an antibody against ADAMTS13, and enzyme
activity was <5%. Immediate plasmapheresis treatment was initiated, followed by plasma
infusions, and again plasmapheresis. A male neonate was delivered by caesarean section
in the 32(nd )week of gestation. The child had an uncomplicated postnatal development.
After delivery, the mother's platelet count and ADAMTS13 activity increased to normal
values. This case shows interesting aspects of TTP in pregnancy and a close cooperation
between obstetricians, nephrologists and pediatricians is necessary for a successful
outcome of the pregnancy.
Source: MEDLINE
85. Isolated thrombocytopaenia: not always idiopathic.
Author(s): Dartey W, Halawa S, Fox R
50
Citation: Journal of Obstetrics & Gynaecology, February 2009, vol./is. 29/2(143), 01443615;1364-6893 (2009 Feb)
Publication Date: February 2009
Source: MEDLINE
Full Text:
Available in fulltext at EBSCO Host
86. Therapeutic approaches to secondary immune thrombocytopenic purpura.
Author(s): Bussel JB
Citation: Seminars in Hematology, January 2009, vol./is. 46/1 Suppl 2(S44-58), 00371963;0037-1963 (2009 Jan)
Publication Date: January 2009
Abstract: Secondary thrombocytopenia is similar to primary or idiopathic
thrombocytopenia (ITP) in that it is characterized by reduced platelet production or
increased platelet destruction resulting in platelet levels<60,000/microL.
Thrombocytopenia can occur from secondary causes associated with chronic disorders or
with disturbed immune function due to chronic infections, lymphoproliferative and
myeloproliferative disorders, pregnancy, or autoimmune disorders. Diagnosis of
secondary ITP in some cases is complex, and the thrombocytopenia can often be resolved
by treating the underlying disorder to the extent this is possible. In most cases, treatment
is focused on reducing platelet destruction, but, in some cases, treatment may also be
directed at stimulating platelet production. The most problematic cases of
thrombocytopenia may be seen in pregnant women. This review will address various
agents and their utility in treating ITP from secondary causes; in addition,
thrombocytopenia in pregnancy, ITP in immunodeficiency conditions, and drug-induced
thrombocytopenia will be discussed. Unlike primary ITP, treatment often must be tailored
to the specific circumstance underlying the secondary ITP, even if the condition itself is
incurable.
Source: MEDLINE
87. Thrombotic microangiopathy in pregnancy.
Author(s): D'Angelo A, Fattorini A, Crippa L
Citation: Thrombosis Research, 2009, vol./is. 123 Suppl 2/(S56-62), 0049-3848;0049-3848
(2009)
Publication Date: 2009
Source: MEDLINE
88. Antenatal intravenous immunoglobulin in chronic immune thrombocytopenic
purpura: case report and literature review.
Author(s): Howman RA, Barr AL, Shand AW, Dickinson JE
Citation: Fetal Diagnosis & Therapy, 2009, vol./is. 25/1(93-7), 1015-3837;1421-9964
(2009)
Publication Date: 2009
Abstract: Immune thrombocytopenic purpura (ITP) may complicate pregnancy and,
uncommonly, may cause severe neonatal thrombocytopenia. However, it is difficult to
predict which neonates are at risk of severe thrombocytopenia. Direct fetal sampling is
51
not commonly done, as it poses significant risks to the fetus. Furthermore, appropriate
antenatal treatment of neonates is controversial. We describe the case of a 32-year-old
woman with chronic severe ITP and a previous severely affected infant, pregnant with
trichorionic triplets, who was successfully managed with the use of weekly intravenous
immunoglobulin 1 g/kg without recourse to direct fetal sampling. (c) 2009 S. Karger AG,
Basel.
Source: MEDLINE
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89. Medical treatments for idiopathic thrombocytopenic purpura during pregnancy.
Author(s): Marti-Carvajal AJ, Pena-Marti GE, Comunian-Carrasco G
Citation: Cochrane Database of Systematic Reviews, 2009, vol./is. /4(CD007722), 13616137;1469-493X (2009)
Publication Date: 2009
Abstract: BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a common
hematologic disorder caused by immune-mediated thrombocytopenia. The magnitude of
the maternal-fetal risk of ITP during pregnancy is controversial. Labour management of
pregnant women with ITP remains controversial. Management of ITP during pregnancy is
complex because of the disparity between maternal and fetal platelet counts.OBJECTIVES:
To assess the effectiveness and safety of corticosteroids, intravenous immunoglobulin,
vinca alkaloids, danazol, dapsone, and any other types of pharmacological treatments for
the treatment of idiopathic thrombocytopenic purpura during pregnancy.SEARCH
STRATEGY: We searched the Cochrane Pregnancy and Childbirth Group's Trials Register
(February 2009), LILACS (1982 to 8 February 2009), ClinicalTrials.gov (8 February 2009),
Current Controlled Trials (16 February 2009), Google Scholar (16 February 2009) and
ongoing and unpublished trials cited in the reference lists of relevant articles.SELECTION
CRITERIA: Randomised controlled trials (RCTs) on any medical treatments for idiopathic
thrombocytopenia purpura during pregnancy.DATA COLLECTION AND ANALYSIS: Two
review authors independently evaluated methodological quality and extracted trial data.
Any disagreement was resolved by discussion or by consulting a third review author.MAIN
RESULTS: This review included one RCT in which 38 women (41 pregnancies) were
randomised, with only 26 women (28 pregnancies) being analysed.This RCT comparing the
effect of betamethasone (1.5 mg/day) with no medication found no statistically significant
difference in neonatal thrombocytopenia (risk ratio (RR) 1.12, 95% confidence interval (CI)
0.62 to 2.05) and neonatal bleeding (RR 1.00, 95% CI 0.24 to 4.13). Review authors
conducted an intention-to-treat analysis which showed similar findings: RR 1.18, 95% CI
0.57 to 2.45 and RR 1.05, 95% CI 0.24 to 4.61, respectively. Maternal death, perinatal
mortality, postpartum haemorrhage and neonatal intracranial haemorrhage were not
studied by this RCT.AUTHORS' CONCLUSIONS: Current evidence indicates that compared
to no medication, betamethasone did not reduce the risk of neonatal thrombocytopenia
and neonatal bleeding in ITP during pregnancy. There is insufficient evidence to support
the use of betamethasone for treating ITP. This Cohrane review does not provide evidence
about other medical treatments for ITP during pregnancy. This systematic review also
identifies the need for well-designed, adequately powered randomised clinical trials for
this medical condition during pregnancy. Unless randomised clinical trials provide
evidence of a treatment effect and the trade off between potential benefits and harms
are established, policy-makers, clinicians, and academics should not use betamethasone
for ITP in pregnant women. Any future trials on medical treatments for treating ITP during
pregnancy should test a variety of important maternal, neonatal or both outcome
measures, including maternal death, perinatal mortality, postpartum haemorrhage and
52
neonatal intracranial haemorrhage.
Source: MEDLINE
Full Text:
Available in fulltext at Wiley
90. [Therapeutic approach in pregnant women with an autoimmune
thrombocytopenic purpura].
Author(s): Christova R, Lisichkov T, Chernev T
Citation: Akusherstvo i Ginekologiia, 2009, vol./is. 48/6(53-4), 0324-0959;0324-0959
(2009)
Publication Date: 2009
Abstract: The case presented herein aim to update the existing information about the
common diagnostic problems and therapeutic approach in pregnant women that have
autoimmune thrombocytopenic purpura (ATP).
Source: MEDLINE
91. [Autoimmune thrombocitopenic purpura in pregnancy].
Author(s): Christova R, Lisichkov T, Chernev T
Citation: Akusherstvo i Ginekologiia, 2009, vol./is. 48/6(42-6), 0324-0959;0324-0959
(2009)
Publication Date: 2009
Abstract: The author deals with haematologists' and obstetricians' current views on
acquired ATP in children and adults, characterised by a transient, acute or chronic
decrease in platelets count (<50.109/l) due to premature destruction by the
reticuloendothelial system. The most common questions arising in connection with this
disease are: what is autoimmune thrombocytopenic purpura; is there any correlation
between pregnancy and ATP; what are its symptoms; does pregnancy itself affect the
autoimmune disease. If is of utter importance for women with ATP to be aware of the
risks these symptoms pose both on the health of the mother and the foetus. Obstetricians
and gynaecologists seldom object to pregnancy in women with ATP. Nevertheless, it is
essential to point out that additional monitoring and therapy are needed. There is no
medical evidence that supports the notion of terminating pregnancy due to ATP.
Assessment is made only by an obstetrician, haematologist and pediatrician working in
close collaboration. This collaborative work must be present throughout the whole
pregnancy, delivery and puerperium. The treatment necessary for women with ATP aims
to establish platelet count over 50.000 ppm when approaching the end of pregnancy,
preferably between 80.000 ppm - 100.000 ppm taking into account vagina or surgical
delivery as well as the administration of anaesthetic. Delivery management must be
decided entirely on obstetrics consideration, but not on ATP ones. Pregnant women with
ATP must be monitored and treated with caution by a highly specialised medical team.
Source: MEDLINE
92. ADAMTS13 deficiency, an important cause of thrombocytopenia during
pregnancy.
Author(s): Kato R, Shinohara A, Sato J
Citation: International Journal of Obstetric Anesthesia, January 2009, vol./is. 18/1(73-7),
0959-289X;1532-3374 (2009 Jan)
53
Publication Date: January 2009
Abstract: We present the case of a woman with congenital ADAMTS13 deficiency and
discuss peripartum management of her fourth pregnancy. All four pregnancies were
complicated by significant thrombocytopenia. Her first pregnancy ended with fetal demise
ascribed to HELLP syndrome and placental abruption. During her second pregnancy, she
was diagnosed with idiopathic thrombocytopenic purpura. Thrombotic thrombocytopenic
purpura and congenital ADAMTS13 deficiency were diagnosed during the third pregnancy.
She had recurrent thrombotic thrombocytopenic purpura during the fourth pregnancy
and responded to treatment with fresh frozen plasma, with a successful outcome. The
need for accurate diagnosis to ensure appropriate treatment is emphasized.
Source: MEDLINE
EMBASE results
1. Fetal/neonatal alloimmune thrombocytopenia (FNAIT)
Author(s): Husebekk A.
Citation: Vox Sanguinis, December 2011, vol./is. 101/(1), 0042-9007 (December 2011)
Publication Date: December 2011
Abstract: Thrombocytopenia is detected in around one percent of newborns. In otherwise
healthy term newborns, thrombocytopenia is most often caused by alloantibodies
transferred from the mother to the foetus. Placental transfer of IgG There is an active
transfer of IgG antibodies from the mother to the foetus based on FcRn receptors on
throphoblasts. The obvious benefit for the foetus is protection from infections in utero
and in the first period after birth. The transfer is by no means based on the specificity of
the IgG molecules and in case of maternal reacting with antigens on foetal cells and
tissues, these are also transferred. Anti-platelet antibodies Platelets have many
polymorphic surface molecules. The most polymorphic are the HLA class I molecules. In
addition, there are polymorphisms in the glycoproteins; altogether 17 biallelic systems of
human platelet proteins (HPA) have been described. The amino acid differences are based
on single nucleotide polymorphisms. Alloimmunization may take place during pregnancy
or upon blood transfusions. The most immunogenic is the HPA-1a epitope. Since the
genetic background differs among ethnic groups, the pattern of immunization differs. In
China and Japan, almost all individuals are HPA-1a and alloimmunization with this antigen
is almost absent. Whereas the HPA-4b antigen is absent in Western countries, it is more
frequently found in Japan and China and immunization with the HPA-4a antigen takes
place. Fetal/neonatal alloimmune thrombocytopenia (FNAIT) FNAIT is most often
diagnosed after birth of a child with thrombocytopenia and bleeding symptoms.
Alloimmune thrombocytopenia is found in 1:1000-2000 newborn, around 30% of the
newborns have severe thrombocytopenia (<50x10E9/l) and may have petecchia and
echymosis. Intracranial haemorrhage (ICH) is seen in 1:12,500-25,000 newborn.
Prospective studies have shown that immunization may take place during pregnancy
(around 25%) or at the time of delivery (75%). Also, prospective studies have shown a
correlation between the maternal antibody level and the severity of thrombocytopenia in
the newborn. Follow-up and treatment of FNAIT No country has introduced general
screening for HPA alloimmunization during pregnancy. In most cases, clinical follow-up
and treatment take place in the subsequent pregnancy after birth of a child with FNAIT.
The mother is given immunomodulatory treatment with intravenous immunoglobulin
(IVIg) and/or steroids. In many cases, delivery is performed by elective caesarean section
and compatible platelet concentrates are available for immediate transfusion to the
newborn if needed. Intrauterine blood sampling and transfusions are no longer part of
54
standard follow-up procedure due to reports of severe complications. Future
management of FNAIT Results from prospective studies have shown that FNAIT is more
similar to haemolytic disease of the newborn (HDN) than thought before. Also, treatment
with IVIg in the pregnancy and careful delivery and transfusion of platelet to the newborn,
reduce morbidity and mortality of FNAIT. Several reports conclude that general screening
should be introduced. Also, there are studies of prophylaxis (anti-HPA-1a antibodies)
aiming at preventing immunization. In the future, FNAIT may be managed similar to HDN
although immunization in early pregnancy cannot be prevented by prophylaxis.
Source: EMBASE
2. Early relapse of thrombotic thrombocytopenic purpura(TTP)/hemolytic uremic
syndrome (HUS) refractory to plasma exchange associated with catheter related
acinetobacter baumannii bacteremia
Author(s): Chang J.W., Tsai C.S., Lin T.H., Hsieh H.H., Tsai Y.U., Lu K.M.
Citation: Vox Sanguinis, December 2011, vol./is. 101/(117-118), 0042-9007 (December
2011)
Publication Date: December 2011
Abstract: Abstract: Thrombotic thrombocytopenic purpura (TTP)/Hemolytic-uremic
syndrome (HUS) is a syndrome characterized by thrombocytopenia, microangiopathic
hemolytic anemia, fever, neurologic manifestation and renal failure. Most of the cases are
idiopathic. Plasma exchange is one of the standard treatment for TTP/HUS. However,
secondary TTP/HUS associated with bacterial, viral and mycobacterial infections, drugs,
autoimmune disease, pregnancy, solid tumors and bone marrow transplantation have
been described. Early relapse associated with catheter-related bacteremia is a rare
occurrence. The patient we report had a classic presentation of TTP/HUS that responded
to plasma exchange but relapsed earlier as reflected by the increased schistocytosis,
decreased hematocrit, decreased platelet counts and increased lactate dehydrogenase.
This relapse may be attributed to Acinetobacter baumannii bacteremia, secondary to
chemo-port infection. After removal of the chemo-port, the syndromes of TTP/HUS had
improved without additional plasma exchange. The importance of identifying the possible
bacterial colonization of an indwelling catheter is thus emphasized.
Source: EMBASE
3. Thrombocytopenia in a girl with idiopathic central precocious puberty treated with
longterm gonadotropin hormone agonists (GnRHa)
Author(s): Krstevska-Konstantinova M., Janchevska A., Gucev Z.
Citation: Hormone Research in Paediatrics, October 2011, vol./is. 76/(268), 1663-2818
(October 2011)
Publication Date: October 2011
Abstract: Background: Central precocious puberty (CPP) is a frequent endocrine problem
in childhood. The idiopathic and organic etiology of CPP are most commonly treated with
GnRH agonists. They have been considered in many studies to be safe and effective. Case
report: We present a 7 year old girl with idiopathic CPP diagnosed by standard GnRH
testing. She developed severe thrombocitopenia during GnRH treatment. The familial
history showed that the mother has been treated 8 years for infertility. The pregnancy
was controlled and uneventful. At the time of diagnosis her weight was at the 75th
percentile and she was 130.5 cm tall on the 97th percentile. The Tanner stage was B3, A1,
P1. The bone age was advanced to 8.5 years. Before the treatment she was otherwise
healthy. All laboratory evaluations were normal from blood count to thyroid function and
brain imaging thehnicques. After receiving her 9th monthly depot therapy of GnRH
55
agonist, triptorelin acetate, 3,75 mg i.m., she developed bleeding from the injection site,
bruises and rush on the skin all over her body. Her platelets were 27 x 103/mul (150-300 x
103/mul). The coagulation factors and myelogram were normal. She was hospitalized at
the haematology department in our hospital for 5 days and treated with corticosteroids.
Recovery was after one week and treatment with GnRH agonists was discontinued.
Conclusions: To our knowledge, thrombocitopenia has not been yet reported in children
receiving GnRH agonist treatment. This may represent a possible serious adverse effect
which needs further investigation.
Source: EMBASE
4. Successful rituximab treatment of refractory immune thrombocytopenia during
pregnancy
Author(s): Schmid J., Piroth D., Buhrlen M., Maass N., Brummendorf T.H., Galm O.
Citation: Onkologie, September 2011, vol./is. 34/(240), 0378-584X (September 2011)
Publication Date: September 2011
Abstract: Immune thrombocytopenia (ITP) is mediated by autoantibodies resulting in
accelerated platelet destruction. ITP clinically manifests in bleeding and can present with
minor symptoms as petechiae, but may also lead to severe intracranial hemorrhage. ITP
with severe thrombocytopenia < 50.000/microL may occur in women during pregnancy
representing a challenge in terms of management and treatment. If corticosteroids and
IVIG (intravenous immungloblin) are not successful, administration of rituximab may be
considered. However, since the IgG-antibody rituximab potentially crosses the placenta,
binding to fetal peripheral B-lymphocytes may subsequently cause immunosuppression.
The following case report describes the effects of rituximab given to a pregnant woman
with ITP after corticosteroids and IVIG had failed to durably increase the platelet count.
Furthermore, reduction of corticosteroids was necessary owing to fetal macrosomy.
Rituximab treatment was initiated in the 25th week of pregnancy and was given weekly
four times (375 mg/m2) leading to a transient increase in maternal platelet count. There
occurred no bleeding complications during and after primary cesarean section. In the
mother platelet count was already normalized three days post partum. In the neonate,
rituximab caused complete B-lymphocyte depletion. B-lymphocyte count was normalized
at two months after delivery, whereas immunoglobulin levels were still inadequate at the
age of 11 months. However, there occurred no complications related to infection in the
neonate. The clinical course indicates that rituximab treatment during pregnancy is
feasible. Administration of rituximab during pregnancy should be carefully considered for
selected cases.
Source: EMBASE
5. Acute kidney injury in pregnancy: The thrombotic microangiopathies
Author(s): Ganesan C., Maynard S.E.
Citation: Journal of Nephrology, September 2011, vol./is. 24/5(554-563), 1121-8428;17246059 (September-Octember 2011)
Publication Date: September 2011
Abstract: Acute kidney injury (AKI) is a rare but serious complication of pregnancy.
Although prerenal and ischemic causes of AKI are most common, renal insufficiency can
complicate several other pregnancy-specific conditions. In particular, severe
preeclampsia/HELLP syndrome, acute fatty liver of pregnancy (AFLP) and thrombotic
thrombocytopenic purpura (TTP) are all frequently complicated by AKI, and share several
clinical features which pose diagnostic challenges to the clinician. In this article, we discuss
the clinical and laboratory features, pathophysiology and treatment of these 3 conditions,
56
with particular attention to renal manifestations. It is imperative to distinguish these
conditions to make appropriate therapeutic decisions which can be lifesaving for the
mother and fetus. Typically AFLP and HELLP improve after delivery of the fetus, whereas
plasma exchange is the first-line treatment for TTP.
Source: EMBASE
Full Text:
Available in fulltext at EBSCO Host
6. Thrombotic thrombocytopenic purpura: Overview on the last 10 years of plasma
exchange treatment in hospital So Jose, Lisbon
Author(s): Santos A., Guttierrez M.J., Tavares M.L.
Citation: Vox Sanguinis, July 2011, vol./is. 101/(296), 0042-9007 (July 2011)
Publication Date: July 2011
Abstract: Introduction Thrombotic Thrombocytopenic Purpura (TTP) was first described by
Moschowitz in 1924. It is a rare disease. In the era before therapeutic plasma exchange
(PE) 90% of patients died from systemic microvascular thrombosis. PE removes the
causative antibody to von Willebrand factor cleaving metalloprotease (ADAMTS13) and
replaces it. Recognition of TTP can be difficult because of the variety of presentations and
lack of specific diagnosis criteria. A diagnosis of TTP may be made in the presence of a
microangiopathic haemolytic anaemia and thrombocytopenia in the absence of any other
identifiable cause. Material and methods: Patient's Hospital medical records were
reviewed since January 2000 until December 2010. Results: In the last ten years we
performed PE in fourteen adult patients (eleven females and three males). Twenty-one
episodes of TTP were observed: one was related to HIV infection and four were related
with first trimester pregnancy. Clinical presentation was heterogeneous and differential
diagnosis was almost exclusively made with acute leukaemia. The most frequent symptom
was mucocutaneous bleeding. Daily PE with replacement of 1.0 to 1.5 times the predicted
plasma volume of the patient was performed, for a minimum of two days after platelet
count and lactate desidrogenase returned to normal. CobeSpectra (Caridian) was used;
the replacement fluid administrated was solvent/detergent-treated plasma (Octaplas,
Octapharma); central venous access was used in all patients. Adjuvant corticosteroid
therapy was instituted. PE was effective in all but one episode. Four patients relapsed.
Four patients were submitted to immunosuppressive agents (rituximab, azatioprin and
cyclophosphamid) when exacerbations or relapse occurred. No measurements of
ADAMTS13 activity or antibody were made. Complications associated with PE were minor
(allergic reaction and high blood pressure). Conclusions: PE is the only treatment for which
there are firm data on its effectiveness in TTP in adults. A high index of suspicion is
required for rapid diagnosis and prompt initiation of PE treatment. PE should be instituted
within 24 hours of presentation of TTP. Plasma infusion remains appropriate when there
may be a delay until PE is available. The optimal duration of therapy is unknown and once
a patient is in remission the efficacy of any treatment to prevent relapses is uncertain.
Source: EMBASE
7. Towards immunological understanding of foetal/neonatal alloimmune
thrombocytopenia based on a study of more than 100,000 pregnancies?
Author(s): Husebekk A., Skogen B., Ahlen M.T., Eksteen M., Heide G., Killie M.K., Killie
I.M.L., Kjeldsen-Kragh J., Ni H., Stuge T.
Citation: Vox Sanguinis, July 2011, vol./is. 101/(37), 0042-9007 (July 2011)
Publication Date: July 2011
57
Abstract: Background: The most common immune responses in transfusion medicine and
in incompatible pregnancies are immune responses to the RhD antigen on red cells, HLA
class I molecules on white cells and platelets, and human platelet antigens. The structure
of these antigens is different; more than 30 epitopes have been detected on the RhD
antigens, HLA antigens are highly polymorphic and induce strong alloimmune responses
whereas the HPA antigens are created by one amino acid difference in allotypes based on
a single nucleotide polymorphism at the genetic level. In Caucasians, HPA-1a induces
immune response and antibody production in 10% of HPA 1b homozygous women in
connection with an HPA-1 incompatible pregnancy. Maternal anti-HPA-1a antibodies of
IgG class cross placenta, bind to foetal HPA-1a positive platelets which are phagocytosed.
The thrombocytopenia renders the foetus/newborn at risk of haemorrhage. Data from
screening of more than 100,000 pregnancies made it possible to understand the biology of
FNAIT in more detail. Aim: The aim of the study was to understand the mechanism of
HPA-1a immunisation and to propose methods for prevention of immunisation and
treatment of women who are already immunised. Methods: Both T cells, B cells and antiHPA-1a antibodies were isolated from HPA-1a alloimmunised women. HPA-1a specific T
cells were isolated after sorting and stimulation with relevant antigen, presented by cells
with HLA DRB30101 HLA class II molecules. Monoclonal anti-HPA-1a IgG was generated by
immortalization of memory B cells. The IgG fraction was isolated from plasma from
women with high level of anti-HPA-1a antibodies. The anti-HPA-1a-mediated immune
suppression was studied in a murine FNAIT model. Results: Analysis of 100,448
pregnancies showed that 2.1% of the pregnant women were homozygous HPA 1bb and
10.6% had detectable antibodies after HPA 1 incompatible pregnancies. It was also shown
that approximately 25% of the women were immunised during the first incompatible
pregnancy and 75% in connection with delivery. Alloimmunised women with blood group
A gave birth to babies with the most severe thrombocytopenia. Both HPA-1a specific
clonal T cells and B cells were isolated from immunized women. The clonal T cells could be
stimulated both by peptides and native antigens provided HLA DRB30101 positive antigen
presenting cells. Studies in mice showed that antibody mediated immune suppression
(AMIS) could be induced and clinical complications prevented if platelet specific
antibodies were injected in connection with immunisation. Summary/conclusions: The
study shows that the pathophysiology of FNAIT and haemolytic disease of the newborn
(HDN) are more similar than believed so far. In HPA 1bb, HLA DRB30101 women with
blood group A, the anti-HPA-1a antibodies induce more severe thrombocytopenia in the
babies compared with mothers with other blood types. The level of maternal antibodies
correlates with the severity of thrombocytopenia in the newborn. There is evidence, in
murine studies, for an AMIS effect of anti-HPA-1a antibodies injected at the time of
immunisation which means that a prophylactic approach may be efficient in preventing
immunisation.
Source: EMBASE
8. New insights in fetal and neonatal alloimmune thrombocytopenia
Author(s): Cecile K., Gerald B.
Citation: Vox Sanguinis, July 2011, vol./is. 101/(35-37), 0042-9007 (July 2011)
Publication Date: July 2011
Abstract: During the recent years considerable advances in the clinical and laboratory
diagnosis of alloimmune thrombocytopenia have been done. Feto-maternal
alloimmunization is the commonest cause of cause of severe isolated fetal and neonatal
thrombocytopenia. The condition results from maternal immunization against specific
fetal platelet antigens (HPA). Unlike the hemolytic disease of the fetus and newborn the
first newborn was found to be affected. The diagnosis is usually made at birth when an
otherwise well term infant exhibits bleeding at delivery or few hours afterwards. The most
58
feared complication of this disorder is the occurrence of intracranial hemorrhage (ICH) as
a result of severe thrombocytopenia leading to death or neurological sequelae. The
diagnosis of alloimmune thrombocytopenia enables appropriate management of the
index case and future pregnancies. The diagnosis is confirmed by laboratory testing with
identification of the maternal alloantibody and the offending antigen present in the fetus
or neonate that is absent in the mother. In a recent retrospective study concerning 75
HPA-1b1b women we have shown that the diagnosis of maternal alloimmunization was
mostly established at delivery and the women were primigravida in 51% of the cases. The
deleterious consequence of this severity was evidenced by in utero or post-natal ICH.
Subsequent pregnancies were managed according to three different protocols, steroids
only, IVIG or IVIG and steroids. We have found that the most efficacious antenatal therapy
for fetal alloimmune thrombocytopenia is maternal treatment with IVIG and steroids. In
summary, this study shows (1) that the morbidity linked with this condition is important to
be considered for further antenatal screening (2) that antenatal management in referral
centers should be suggested to high-risk pregnant women. Introduction: Fetal and
neonatal alloimmune thrombocytopenia (FNAIT) resulting from maternal immunization
against specific fetal platelet antigen [1] affects the fetus early during pregnancy[2]. This
syndrome as been considered as the platelet counterpart of hemolytic disease of the
neonate (HDN) but in contrast to HDN, retrospective studies have shown that the first
pregnancy was frequently affected[3]. The fetus has long been considered as an innocent
bystander. However recent studies in a Mouse model have shown that the fetal major
histocompatibility complex class I related neonatal Fc receptor (FcRn) is implicated in the
transfer of maternal alloantibodies[4]. FNAIT is the most common cause of severe isolated
fetal/neonatal thrombocytopenia, which the most feared complication is the occurrence
of intracranial hemorrhage (ICH) leading to death or neurological sequelae. The incidence
of FNAIT has been estimated to 1/800 to 1/1000 live births in Caucasians. However
because of the absence of clinical bleeding in moderate thrombocytopenia, these cases
would be overlooked in the absence performing routine screening. Clinical presentation:
Fetal thrombocytopenia has been defined in as a platelet count less than 150x109/L,
irrespective of the gestational age[5]. Fetal thrombocytopenia is often discovered when
ICH has occurred. Sonography reveals ventriculomegaly or fetal hydrocephalus. ICH has
been documented whatever the implicated platelet alloantigens, (25.5% of cases for HPA1a, 24% for HPA-3a, 15% for HPA-5b)[6], leading to death in up to 10% or neurological
sequelae in up to 20% of the reported cases. In a literature review it has been reported
that 80% of ICH occur in utero and 40% are diagnosed before 30 weeks of gestation[6].
Most commonly, neonatal thrombocytopenia is suspected in otherwise well term infant
with unexplained bruising and purpura. In some circumstances, severe bleeding as ICH,
large cephalohematoma, gastrointestinal or genitourinary hemorrhage is observed. In any
case a platelet count should be obtained. The risk of life-threatening hemorrhage in case
of severe thrombocytopenia necessitates prompt diagnosis and therapy. Conversely the
infant may be symptomless with thrombocytopenia discovered incidentally when a blood
count is obtained for another reason i.e. to exclude sepsis. Our recent retrospective study
shows that the diagnosis of maternal platelet alloimmunization in our cohort was
established either during the pregnancy (9 women) or at delivery (66 women)[7]. In this
series the pregnant women were primigravida in 51% of the cases. The cases referred to
the laboratory for investigation were severe with 6 ICH detected antenatally while three
cases were diagnosed in the post-natal period, leading to death for two neonates. The
severe thrombocytopenia in our index cases (80% of the neonates with a platelet count
below 50 x109/L) was not correlated either with the maternal alloantibody concentration
at delivery, or the maternal genetic background (ABO or HLA). It is important to point out
that eight neonates were born before the diagnosis of FNAIT: six infants had no medical
problem despite the HPA-1 feto-maternal incompatibility (no platelet count performed at
the time), and two were thrombocytopenic but the FNAIT diagnosis was not done at the
time. Therefore, unexpected or unexplained neonatal thrombocytopenia or severe early59
onset thrombocytopenia should raise the possibility of FNAIT and guide investigations
accordingly. Laboratory testing for suspected FNAIT: The diagnosis of FNAIT is important:
(1) for the management of the index case, and (2) for the antenatal management of
subsequent pregnancies. The laboratory diagnosis relies on the detection and
identification of the maternal antiplatelet antibody, and identification of the offending
antigen. Investigation should be performed by an experienced laboratory that has the
ability to perform antigencapture assays for antibody testing, and typing of common
HPAs, and even rare or new platelet alloantigens. To date, 27 platelet-specific alloantigens
have been described, 12 with a bi-allelic polymorphism[8] (http://www.
ebi.ac.uk/ipd/hpa/). Frequencies of platelet antigens vary among different populations. In
Caucasians, HPA-1a is by far the most common antigen implicated in FNAIT, followed at
much lower frequency by HPA-5b, then HPA-3. In contrast, in Asians, FNAIT is essentially
linked with HPA-4 and HPA-5b. During recent years FNAIT has been reported involving
rare or private antigens, most of them located on the GPIIb-IIIa complex. The description
of such antigens has been possible with the introduction of a routine maternal-paternal
cross-matching with an antigen-capture assay and a panel of mouse monoclonal
antibodies in investigations of suspected cases of FNAIT. Recent studies have shown these
low-frequency antigens being not restricted to single families [9-11]. Detection and
identification of the causative maternal alloantibody is done by an Elisa antigen-capture
assay, and the MAIPA (Monoclonal Antibody-specific Immobilization of Platelet Antigens
[12]) is considered the gold standard reference method in platelet immunology. Platelet
phenotyping may be performed with the MAIPA technique, although due to shortage of
serological reagents and advance in molecular biology, platelet genotyping is usually done.
The recent development of high-throughput technologies allows simultaneous genotyping
of as many as 17 HPAs. However genotype is not phenotype. Discrepancies due to
unknown mutations may alter the technique and may be responsible for false typing
assignation with potential consequences on diagnosis and therapy [11,13]. For that reason
we recommend the use of both phenotyping and genotyping for at least the most
frequently involved platelet antigens. Post-natal management: Severely affected infants
with bleeding or a platelet count <30.109/L during the first 24 hours of life should be
promptly transfused with compatible platelets. The mother has been considered as the
best donor, but logistic problems in emergency situation have lead to alternatives such as
provision of frozen-thawed platelet concentrates or transfusion of random platelet
concentrates combined with perfusion of intravenous immunoglobulin [14]. Whatever the
situation, it is important to monitor the platelet count to ensure that a safe threshold
(>50x109/L) has been reached and no additional therapy is required. Ultrasound
examination is recommended to exclude ICH. The neonatal outcome in the absence of
bleeding is generally favorable; a normal platelet count is obtained within a week of life.
Antenatal management: As the severity of thrombocytopenia usually increases in
subsequent pregnancies, antenatal management of high-risk pregnancies has been
developed to prevent the morbidity and mortality of this condition.
Source: EMBASE
9. Maternal antibody titration as a predictive parameter for fetal status and therapy
effectiveness in pregnancies associated with alloimmune thrombocytopenia
Author(s): Gerald B., Cecile K.
Citation: Vox Sanguinis, July 2011, vol./is. 101/(34-35), 0042-9007 (July 2011)
Publication Date: July 2011
Abstract: Alloimmune thrombocytopenia is the most common cause of severe isolated
fetal and neonatal thrombocytopenia. In view of the recurrence of thrombocytopenia in
subsequent pregnancies with incompatible foetuses, antenatal management has been
developed. Until recently the only possibility of assessing the fetal status both before and
60
during therapy was to perform fetal blood samplings (FBS). In view of the risks involved in
the procedure, FBS has been restricted and non-invasive strategies have been developed.
The determination of maternal parameters predictive of severe fetal thrombocytopenia is
crucial for tailored intervention. A first retrospective study was designed to analyse the
predictive value of the maternal anti HPA-1a antibody concentration. With this in view, we
developed a quantitative method based on the Monoclonal Antibodyspecific
Immobilization of Platelet Antigens (MAIPA) technique, which is the gold standard method
for serological investigations in platelet immunology (Bertrand et al., Transfusion, 2005).
Maternal anti HPA-1a antibody concentrations were determined at the same time as the
FBS carried out as a part of the antenatal management prior to therapy. A statistically
significant correlation was observed between the high antibody concentration [>=28
International Units (IU) /mL] and severe fetal thrombocytopenia (<50x109/L; P = 0.0021;
Bertrand et al. J Thromb Haemostasis, 2006). A larger retrospective study was
subsequently performed to search for additional maternal predictive parameters during
managed pregnancies, taking into account maternal anti HPA-1a antibody concentrations
and maternal genetic background (ABO blood group and HLA-DRB3 allele). We confirmed
the predictive value of the maternal antibody concentration before 28 wg and before
treatment. The follow-up of the concentration during pregnancy allowed the
measurement of the area under curve, weighted by the weeks between the first and the
last quantification. This new parameter was predictive of the therapy effectiveness: under
24 IU/ mL/wg, a majority of women delivered a severely thrombocytopenic newborn
(>50x109/L; P = 0.0153; Bertrand et al. Blood, in press). In conclusion, our work gives new
insights into maternal predictive parameters for fetal status and therapy effectiveness,
allowing noninvasive strategies. Follow-up of the antibody concentration during
pregnancy could help to predict the outcome of the pregnancy, so as to prevent severe
hemorrhagic disorders in the neonate. Introduction: Alloimmune thrombocytopenia is the
most common cause of severe isolated fetal and neonatal thrombocytopenia. This results
from maternal immunization against fetal platelet-specific antigens inherited from the
father. The frequency of fetal/neonatal alloimmune thrombocytopenia (F/NAIT) is about
1/1000 live births. In Caucasian populations, incompatibility concerning the HPA-1a
antigen plays a major role in F/ NAIT. As the severity of thrombocytopenia usually
increases in subsequent pregnancies, antenatal management of high-risk pregnancies is
very important in preventing the morbidity or mortality linked with severe fetal
thrombocytopenia. Until recently the only possibility of assessing the fetal status both
before and during therapy was to perform fetal blood samplings (FBS). In view of the risks
involved in the procedure, FBS has been restricted and non-invasive strategies have been
developed. The determination of maternal parameters predictive of severe fetal
thrombocytopenia is crucial for tailored intervention. For this purpose, we designed a first
retrospective study to analyse the predictive value of the maternal anti HPA-1a antibody
concentration during and after pregnancy. A larger retrospective study was subsequently
performed to search for additional maternal predictive parameters during managed
pregnancies, taking into account maternal anti HPA-1a antibody concentrations and
maternal genetic background (ABO blood group and HLA-DRB3 allele). The most striking
features of these retrospective studies are presented. Methodology for quantification of
maternal anti HPA-1a antibodies: The Monoclonal Antibody-specific Immobilization of
Platelet Antigens (MAIPA) assay [Kiefel, 1987 179 /id] is the gold standard for the
detection of anti platelet antibody [Kaplan, 2007 1954 /id]. The MAIPA is an
antigencapture assay, based on the formation of trimolecular complexes by the binding of
specific mouse monoclonal antibodies (MoAbs) and the human antibody targeting the
platelet membrane molecule on which the respective epitopes are located. We have
standardized this procedure for the quantification of anti HPA-1a antibodies [Bertrand,
2005 1767 /id]. Eight serial dilutions of a reference serum containing 100 IU/mL of anti
HPA-1a antibodies [Allen, 2005 2075 /id] were performed to construct a standard curve
fitting with a 4-parameter logistic regression (from 1 to 1/128; Figure 1). Test sera were
61
also diluted from 1 to 1/128, and optical density values were used for the interpolation of
the concentration with the standard curve corrected by the dilution factor. This procedure
was followed to determine the concentration of sera from HPA-1bb women, in a context
of F/NAIT. Severe fetal thrombocytopenia and the predictive value of the antibody
concentration: Prospective studies seeking a relationship between maternal alloantibody
concentration and the fetal status have failed to reach a consensus [Bessos, 2005 1786
/id;Durand-Zaleski, 1996 913 /id;Jaegtvik, 2000 1429 / id;Kaplan, 1988 260 /id;Killie, 2010
2143 /id;Kurz, 1999 1721 /id;Proulx, 1994 880 /id;Williamson, 1998 1310 /id]. These
discrepancies may be partly explained by the size of the series, the parity of the women
enrolled, the differences in the timing of maternal sampling, and the methodology used
for the antibody titration. We designed a first retrospective study to determine if there is
a relationship between the maternal anti HPA-1a antibody concentration and the fetal
platelet count. Titrations of 31 maternal sera at different stages of pregnancy [16-37
weeks of gestation (wg)] before any antenatal therapy were determined at the same time
as the FBS carried out as a part of the antenatal management prior to therapy. A
statistically significant correlation was observed between the severely thrombocytopenic
fetuses and antibody concentrations above 28 IU/mL when measured before 28 wg and
before any treatment (Fisher's exact test P = 0.015 [Bertrand, 2006 1849 /id]). A larger
retrospective study was subsequently performed, including 239 pregnancies from 75 HPA1bb women [Bertrand, 2011 2158 /id]. The figure 2A shows fetal platelet counts
determined by FBS, according to the maternal antibody concentrations (x-axis). This
enlarged cohort led to an improvement in the prediction of the fetal status (P = 0.0016),
ROC curve in Figure 2B). Follow-up of the maternal antibody concentration during
pregnancy, and prediction of the therapy effectiveness: A recent meta-analysis from
Bussel et al. showed that about 20% of women delivered a severely thrombocytopenic
newborn despite the administration of immunoglobulin G (IVIG) during managed
pregnancy [Vinograd, 2010 2177 /id]. This percentage was calculated by taking into
account numerous studies supporting the efficacy of IVIG-therapy. In order to prevent
severe post-natal hemorrhagic disorders, it would be of interest to predict the outcome of
the pregnancy. We searched for additional maternal predictive parameters indicating the
severity of the disease, at the time of the diagnosis as well as during subsequent managed
pregnancies (IVIG-treated mothers). We took into account the gynaecologic history of the
women, their genetic background (ABO blood group and HLA DRB3 allele), and we
followed the maternal antibody concentration during the pregnancy of 34 IVIG-treated
women. In our cohort, the severity of thrombocytopenia in index cases was not correlated
with the maternal ABO blood group (P = 0.6599) or the HLA DRB3:0101 allele (P = 0.1664).
Management of subsequent pregnancies relies on (1) an initial determination of the
antibody concentration before 28 wg and before treatment, to determine the severity of
the fetal status and (2) the administration of intravenous IVIG with the follow-up of the
antibody concentration till delivery. The pattern of the antibody concentration during
pregnancy was variable: steadily low antibody concentration, or decreasing towards
delivery, or increasing during the second and third trimester of pregnancy or just after
delivery. In order to analyze these curve tendencies, we measured the area under curve
(AUC) of each antibody follow-up (34 managed pregnancies), and weighted the AUC by
the weeks' gestation between the first and the last quantification. Figure 3 reports an
example of antibody follow-up during the pregnancies of two women, both pregnant for
the second time. The antibody concentration followed during pregnancy A remained very
low (weighted AUC = 6 IU/mL/wg) and the newborn had a normal platelet count. On the
contrary, the antibody concentration was very high during pregnancy B (weighted AUC =
69 IU/mL/wg), and the newborn was severely thrombocytopenic (post-natal therapy
necessitates platelet transfusion associated with IVIG injections). Proceeding with this
methodology for the 34 pregnancies, our study showed that this new maternal parameter
is predictive of the therapy failure: under 28 IU/mL/wg, a majority of newborns were
below the safe platelet count of 50x109/L (P = 0.0153).
62
Source: EMBASE
10. Successful management of a planned pregnancy in severe congenital thrombotic
thrombocytopaenic purpura: The Upshaw-Schulman syndrome
Author(s): Richter J., Strandberg K., Lindblom A., Strevens H., Karpman D., Wide-Swensson
D.
Citation: Transfusion Medicine, June 2011, vol./is. 21/3(211-213), 0958-7578;1365-3148
(June 2011)
Publication Date: June 2011
Source: EMBASE
11. Use of fondaparinux in a pregnant woman with pulmonary embolism and heparininduced thrombocytopenia
Author(s): Ciurzynski M., Jankowski K., Pietrzak B., Mazanowska N., Rzewuska E., Kowalik
R., Pruszczyk P.
Citation: Medical Science Monitor, May 2011, vol./is. 17/5(CS56-CS59), 1234-1010;16433750 (May 2011)
Publication Date: May 2011
Abstract: Background: A serious complication of heparin treatment, heparin-induced
thrombocytopenia (HIT) is rarely observed in pregnant women. Drug therapy during
pregnancy should always be chosen to minimize fetal risk. The management of HIT in
pregnancy represents a medical challenge. Unlike heparins, the anticoagulants used in
patients with HIT do cross the placenta, with unknown fetal effects. Case Report: We
present a case of a 24-year-old female presenting for care at 34 weeks of gestation with
acute pulmonary embolism treated initially with unfractionated heparin (UFH) and low
molecular weight heparin (LMWH), who developed HIT. She was then successfully treated
with fondaparinux. Conclusions: To the best of our knowledge, this is one of the first case
reports describing a successful use of fondaparinux in the treatment of HIT in a thirdtrimester pregnant woman, providing a novel approach for this subset of patients. Med
Sci Monit, 2011.
Source: EMBASE
12. Diagnosis and management of thrombocytopenia in pregnancy
Author(s): Myers B., Truelove E.
Citation: Fetal and Maternal Medicine Review, May 2011, vol./is. 22/2(144-167), 09655395;1469-5065 (May 2011)
Publication Date: May 2011
Abstract: In conclusion, there are a large number of causes of thrombocytopenia in
pregnancy. By far the most common causes are mild, but awareness of the complex
disorders in which thrombocytopenia occurs is essential to ensure prompt diagnosis and
referral into a centre with expertise in these rare conditions, to optimise outcome for
mother and baby. 2011 Cambridge University Press.
Source: EMBASE
13. The American Society of Hematology 2011 evidence-based practice guideline for
immune thrombocytopenia
Author(s): Neunert C., Lim W., Crowther M., Cohen A., Solberg Jr. L., Crowther M.A.
63
Citation: Blood, April 2011, vol./is. 117/16(4190-4207), 0006-4971;1528-0020 (21 Apr
2011)
Publication Date: April 2011
Abstract: Immune thrombocytopenia (ITP) is commonly encountered in clinical practice. In
1996 the American Society of Hematology published a landmark guidance paper designed
to assist clinicians in the management of this disorder. Since 1996 there have been
numerous advances in the management of both adult and pediatric ITP. These changes
mandated an update in the guidelines. This guideline uses a rigorous, evidence-based
approach to the location, interpretation, and presentation of the available evidence. We
have endeavored to identify, abstract, and present all available methodologically rigorous
data informing the treatment of ITP. We provide evidence-based treatment
recommendations using the GRADE system in those areas in which such evidence exists.
We do not provide evidence in those areas in which evidence is lacking, or is of lower
quality - interested readers are referred to a number of recent, consensus-based
recommendations for expert opinion in these clinical areas. Our review identified the
need for additional studies in many key areas of the therapy of ITP such as comparative
studies of "front-line" therapy for ITP, the management of serious bleeding in patients
with ITP, and studies that will provide guidance about which therapy should be used as
salvage therapy for patients after failure of a first-line intervention. 2011 by The American
Society of Hematology.
Source: EMBASE
Full Text:
Available in fulltext at Highwire Press
14. Thrombocytopenia in Pregnancy
Author(s): Bockenstedt P.L.
Citation: Hematology/Oncology Clinics of North America, April 2011, vol./is. 25/2(293310), 0889-8588 (April 2011)
Publication Date: April 2011
Abstract: Thrombocytopenia in pregnancy is most frequently a benign process that does
not require intervention. However, 35% of cases of thrombocytopenia in pregnancy are
related to disease processes that may have serious bleeding consequences at delivery or
for which thrombocytopenia may be an indicator of a more severe systemic disorder
requiring emergent maternal and fetal care. Thus, all pregnant women with platelet
counts less than 100,000/muL require careful hematological and obstetric consultation to
exclude more serious disorders. 2011 Elsevier Inc.
Source: EMBASE
15. ADAMTS13 activity and the risk of thrombotic thrombocytopenic purpura relapse
in pregnancy
Author(s): Raman R., Yang S., Wu H.M., Cataland S.R.
Citation: British Journal of Haematology, April 2011, vol./is. 153/2(277-279), 00071048;1365-2141 (April 2011)
Publication Date: April 2011
Source: EMBASE
16. Fetal alloimmune thrombocytopenia: Is less invasive antenatal management safe?
64
Author(s): Mechoulan A., Kaplan C., Muller J.Y., Branger B., Philippe H.J., Oury J.-F., Ville Y.,
Winer N.
Citation: Journal of Maternal-Fetal and Neonatal Medicine, April 2011, vol./is. 24/4(564567), 1476-7058;1476-4954 (April 2011)
Publication Date: April 2011
Abstract: Objectives. The aim of this study was to review recent multicenter data on
antenatal management of anti-HPA-1a fetal alloimmune thrombocytopenia and, based on
this retrospective study and on recent literature, to evaluate if FBS modified the
obstetrical management. Material and methods. This retrospective study in France
includes 23 pregnancies in 21 women who had a previous thrombocytopenic infant due to
anti HPA-1a alloimmunization. All pregnant women received intravenous immunoglobulin
(IVIG) treatment, with or without corticosteroids. Fetal blood sampling (FBS) was
performed before any therapy (four cases) or during pregnancy (nine cases). Results.
Infants whose mother received treatment had a significantly higher neonatal platelet
count than the corresponding sibling (p = 0.003). In eight cases, therapy was started late
during pregnancy. In three cases, treatment was discontinued 3 or 4 weeks before birth,
and this was associated with a poorer result. No in utero intracranial hemorrhage was
recorded in the infants for whom maternal therapy continued to term. Adverse effects
were not observed in any case. All babies were delivered by cesarean even when FBS was
performed. One emergency cesarean was performed for fetal bradycardia after FBS.
Conclusion. This study confirmed that maternal therapy with intravenous immunoglobulin
for fetal alloimmune thrombocytopenia gives satisfactory results. It also showed that a
less invasive approach, especially a reduction in the number of fetal blood samples, is
possible without deleterious consequences. This observation suggests also to start IVIG
early during pregnancy and to continue treatment up to delivery. 2011 Informa UK, Ltd.
Source: EMBASE
17. Reversal of thrombocytopenia in a pregnant woman after changing
hemodiafiltration membranes
Author(s): Venditto M., Bourry E., Szumilak D., Deray G.
Citation: American Journal of Kidney Diseases, March 2011, vol./is. 57/3(521), 0272-6386
(March 2011)
Publication Date: March 2011
Source: EMBASE
18. Prediction of the fetal status in noninvasive management of alloimmune
thrombocytopenia
Author(s): Bertrand G., Drame M., Martageix C., Kaplan C.
Citation: Blood, March 2011, vol./is. 117/11(3209-3213), 0006-4971;1528-0020 (17 Mar
2011)
Publication Date: March 2011
Abstract: Fetal/neonatal alloimmune thrombocytopenia is the most common cause of
severe thrombocytopenia in the fetus and in an otherwise healthy newborn. To counter
the consequences of severe fetal thrombocytopenia, antenatal therapies have been
implemented. Predictive parameters for fetal severe thrombocytopenia are important for
the development of noninvasive strategy and tailored intervention. We report here data
concerning 239 pregnancies in 75 HPA-1bb women. Analysis of the index cases (diagnosis
of fetal/neonatal alloimmune thrombocytopenia) did not show any significant correlation
between the severity of the disease and the maternal genetic background (ABO blood
65
group and HLA-DRB3 allele). Subsequent pregnancies were managed, and therapy
effectiveness was evaluated. The highest mean newborn platelet count was observed for
a combination of intravenous immunoglobulin and steroids (135 x 10 9/L; 54 newborns)
compared with intravenous immunoglobulin alone (89 x 109/L; 27 newborns). The
maternal anti-HPA-1a antibody concentration measured before any treatment and before
28 weeks of gestation was predictive of the fetal status. The weighted areas under curves
of the maternal alloantibody concentrations were predictive of therapy response. To
conclude, this large retrospective survey gives new insights on maternal predictive
parameters for fetal status and therapy effectiveness allowing noninvasive strategies.
2011 by The American Society of Hematology.
Source: EMBASE
Full Text:
Available in fulltext at Highwire Press
19. Thrombotic thrombocytopenic purpura with complete molar pregnancy: a case
report
Author(s): Meng H., Kumar N.S., Nannapaneni J., Inglis S.R.
Citation: The Journal of reproductive medicine, March 2011, vol./is. 56/3-4(169-171),
0024-7758 (2011 Mar-Apr)
Publication Date: March 2011
Abstract: Thrombotic thrombocytopenic purpura (TTP) is extremely rare. This disease and
its prompt diagnosis are important because TTP in pregnancy carries a 90% mortality rate.
A 21-year-old woman underwent suction dilation and curettage for molar pregnancy.
Postoperatively the patient developed severe hypertension, microangiopathic anemia,
thrombocytopenia and chest pain associated with ischemic cardiac changes. Despite
blood and plasma transfusions and steroid therapy, the patient continued to have
worsening hemolysis and thrombocytopenia. TTP was diagnosed, and plasmapheresis led
to a rapid recovery. TTP can occur with molar pregnancy. Making this diagnosis in a timely
manner is crucial to ensure that potentially life-saving plasmapheresis is initiated in a
timely manner. To our knowledge, this is the first reported case of thrombotic
thrombocytopenic purpura with molar pregnancy.
Source: EMBASE
20. Immune thrombocytopenic purpura in pregnancy: a reappraisal of obstetric
management and outcome
Author(s): Gasim T.
Citation: The Journal of reproductive medicine, March 2011, vol./is. 56/3-4(163-168),
0024-7758 (2011 Mar-Apr)
Publication Date: March 2011
Abstract: To evaluate the complications of pregnancy and perinatal outcome in women
with idiopathic thrombocytopenic purpura (ITP). A retrospective analysis of 38 singleton
pregnancies, their course, obstetric management and perinatal outcome of 32 patients
with known ITP was undertaken. No major antenatal complications were noted among the
patients. There were no maternal deaths, and only 1 stillbirth occurred in the series.
Fourteen infants were delivered by cesarean section and 24 by vaginal delivery. Neonatal
cord blood platelet count was performed in each of the live-born infants and revealed
thrombocytopenia in 16 infants, but in only 6 (16.2%) of them was the cord blood platelet
count < 50 x 10(9)/L. There was no neonatal death in the study, although 6 infants
required supportive treatment with corticosteroids and intravenous immunoglobulin G.
66
No maternal features could be used to predict the neonatal platelet count at birth. These
results are comparable with other studies in the recent literature. Due to the low
incidence of poor neonatal outcome in mothers with ITP, obstetric intervention based
solely on their platelet count is not justified. Every patient with ITP should be managed
individually, and the routine use of cesarean section should be abandoned.
Source: EMBASE
21. Delayed heparin-induced thrombocytopenia in pregnant patient with APLA:
Diagnosis and treatment
Author(s): Kozak N., Sigler E., Fleisher S., Tomer A.
Citation: Thrombosis Research, February 2011, vol./is. 127/(S134), 0049-3848 (February
2011)
Publication Date: February 2011
Abstract: A 26 y/o patient was diagnosed with APLA syndrome after her first unprovoked
DVT. The laboratory data confirmed the triple positivity, i.e., strongly positive LAC, and
high titers (more than 100) both of anticardiolipin and anti-beta2glycoprotein. The patient
was placed on Warfarin and two months later after becoming pregnant she switched to
therapeutic LMWH. The pregnancy was uneventful up to 32 week when sudden drop in
platelet count (from 287x103 to 70x103 in a week) raised the suspicion of HIT in spite of
unusual timing and context. PaGIA test was positive but considering possible cross
reactivity with APLA we decided to perform the confirmatory functional flow cytometric
assay (FCA) which determines the capacity of the patient's serum to activate platelets in
the presence of Heparin. FCA was also positive. LMWH was stopped and the patient was
placed on Fondaparinux with gradual improvement in platelet count after 4 days of
treatment. She continued on this regimen until regular vaginal delivery when her platelets
were 110x103. Five days after delivery additional increase up to 210x103 enabled her to
restart Warfarin. This case presents a number of special features: successful pregnancy
with very high APLA titers, HIT in pregnancy and after six months of treatment,
combination of HIT and APLA. In addition of note is the application of flow cytometry as
confirmatory diagnostic assay and use of Fondaparinux in HIT in pregnancy with good
results and without any complications.
Source: EMBASE
22. Unusual case of thrombocytopenia with terminal 1Q deletion
Author(s): Syed S.A., Martinez J., Savells K.
Citation: Journal of Investigative Medicine, February 2011, vol./is. 59/2(448), 1081-5589
(February 2011)
Publication Date: February 2011
Abstract: Case Report: Introduction: Terminal deletion of chromosome 1q is a
recognizable entity usually presenting with abnormalities of the CNS and skeletal system.
Hematological abnormalities are however rare. We present the first reported case of 1q
terminal deletion with persistent neonatal thrombocytopenia. A male infant was born via
C-section secondary to multiple fetal anomalies, at 36 weeks gestation to a
nonconsanguineous couple. The pregnancy was complicated by abnormal prenatal
ultrasound that showed polyhydramnios, micrognathia, and a 2 vessel cord. At birth, the
infant was noted to be pale with poor perfusion. Birth weight was 2295grams (10th %),
length 44.5cm (10th %), head cir. 31.5 cm (10th %). Multiple anomalies were present at
birth including short neck, low set dysplastic ears, short upturned nose, micrognathia,
micro-penis with hypospadias, hypoplastic and empty scrotum, puffy hands and digits,
flexion contracture, (claw hands). ECHO showed ventriculoseptal defect (VSD) and large
67
atrial septal defect. Cranial Doppler showed agenesis of the corpus callosum, hypoplastic
cerebellar vermis, and large ventricles communicating with normal-size cisterna magna.
Abdominal ultrasound showed mild bilateral hydronephrosis and small splenic cyst. Labs
at birth showed thrombocytopenia (platelet count of 9) and severe anemia (H/H 5.6/16).
Over the course of NICU stay, anemia resolved after one PRBC transfusion at birth.
However, a thrombocytopenia persisted that required multiple platelet transfusions.
Secondary to good response to platelet transfusion, his problem seems to be related to
platelet production rather than destruction therefore related to a dysplastic bone marrow
function. The infant failed a hearing screen in both ears. Both CMV and toxoplasmosis
were negative. CGH array revealed del (1)(q42.3 or 43->qter) and a dup(2)(q37.3->qter).
Conclusion: The baby was born with most of the clinical features of deletion 1q42
including agenesis of corpus callosum, hypospadias, cardiovascular anomalies, and other
less common manifestations including Cutis marmorata and claw-shaped hands.
Thrombocytopenia has not been previously reported in patients with this deletion which
made our case an unusual one.
Source: EMBASE
23. Thrombocytopenia and disorders of platelet function in pregnancy
Author(s): Kadir R.A., McLintock C.
Citation: Seminars in Thrombosis and Hemostasis, 2011, vol./is. 37/6(640-652), 00946176;1098-9064 (2011)
Publication Date: 2011
Abstract: Pregnancy is associated with physiological and pathological changes in platelet
numbers and function, which can be of clinical concern because of risks for maternal and
fetal or neonatal bleeding. Thrombocytopenia in pregnancy is frequently encountered and
may be due to increased platelet turnover and plasma dilution, immune-mediated
mechanisms, or a complication of a more severe underlying pregnancy-related disorder
such as preeclampsia. Inherited defects in platelet function and number may also manifest
during pregnancy with the risk of bleeding dependent on the underlying problem. In some
women, the diagnosis of thrombocytopenia will precede pregnancy but in others, the
problem is first identified when routine pregnancy blood tests are performed. An accurate
diagnosis and risk assessment in the antenatal period are essential for developing specific
plans for any antenatal interventions and for management of delivery and the postpartum
periods, and the neonate. Management of pregnant women with platelet disorders
requires a multidisciplinary approach and close collaboration between the obstetric and
hematology teams. 2011 by Thieme Medical Publishers, Inc.
Source: EMBASE
24. Antenatal interventions for fetomaternal alloimmune thrombocytopenia
Author(s): Rayment R., Brunskill S.J., Soothill P.W., Roberts D.J., Bussel J.B., Murphy M.F.
Citation: Cochrane database of systematic reviews (Online), 2011, vol./is. 5/(CD004226),
1469-493X (2011)
Publication Date: 2011
Abstract: Fetomaternal alloimmune thrombocytopenia results from the formation of
antibodies by the mother which are directed against a fetal platelet alloantigen inherited
from the father. The resulting fetal thrombocytopenia (reduced platelet numbers) may
cause bleeding, particularly into the brain, before or shortly after birth. Antenatal
treatment of fetomaternal alloimmune thrombocytopenia includes the administration of
intravenous immunoglobulin (IVIG) and/or corticosteroids to the mother to prevent
severe fetal thrombocytopenia. IVIG and corticosteroids both have short-term and
68
possibly long-term side effects. IVIG is also costly and optimal regimens need to be
identified. To determine the optimal antenatal treatment of fetomaternal alloimmune
thrombocytopenia to prevent fetal and neonatal haemorrhage and death. We searched
the Cochrane Pregnancy and Childbirth Group's Trials Register (28 February 2011) and
bibliographies of relevant publications and review articles. Randomised controlled studies
comparing any intervention with no treatment, or comparing any two interventions. Two
review authors independently assessed eligibility, trial quality and extracted data. We
included four trials involving 206 people. One trial involving 39 people compared a
corticosteroid (prednisone) versus IVIG alone. In this trial, where analysable data were
available, there was no statistically significant differences between the treatment arms for
predefined outcomes. Three trials involving 167 people compared IVIG plus a
corticosteroid (prednisone in two trials and dexamethasone in one trial) versus IVIG alone.
In these trials there was no statistically significant difference in the findings between the
treatment arms for predefined outcomes (intracranial haemorrhage; platelet count at
birth and preterm birth). Lack of complete data sets and important differences in
interventions precluded the pooling of data from these trials. The optimal management of
fetomaternal alloimmune thrombocytopenia remains unclear. Lack of complete data sets
for two trials and differences in interventions precluded the pooling of data from these
trials which may have enabled a more developed analysis of the trial findings. Further
trials would be required to determine optimal treatment (the specific medication and its
dose and schedule). Such studies should include long-term follow up of all children and
mothers.
Source: EMBASE
Full Text:
Available in fulltext at Wiley
25. IVIG to prevent fetal intracranial hemorrhage in fetal and neonatal alloimmune
thrombocytopenia (FNAIT): Can we reduce the dose to 0.5 g/kg/wk?
Author(s): Wikman A., Tiblad E., Westgren M., Paridaans N., Kamphuis M., Lopriore E.,
Oepkes D., Van Den Akker E.
Citation: American Journal of Obstetrics and Gynecology, January 2011, vol./is. 204/1
SUPPL.(S32-S33), 0002-9378 (January 2011)
Publication Date: January 2011
Abstract: OBJECTIVE: The standard and highly effective treatment of pregnancies with
FNAIT in which the previous affected child had severe thrombocytopenia but no
intracranial hemorrhage (ICH) is weekly intravenous administration of immunoglobulins
(IVIG) in a dose of 1 g/kg maternal weight. IVIG is an expensive human multidonor
bloodproduct with headaches as main clinical side-effect. Dose-finding studies were never
performed, this dose was based on use of IVIG for other diseases. In vitro studies
suggested that a lower dose could be just as effective. Aim of our study was to compare
the effect of a weekly dose IVIG of 0.5 g/kg with the traditional 1 g/kg. STUDY DESIGN:
International prospective multicenter study from the Huddinge Hospital, Karolinska
Institute, Stockholm, Sweden and The Leiden University Medical Center, The Netherlands.
Pregnant women with HPA-1a or 5b alloantibodies against fetal platelets, a fetus positive
for the antigen and an affected sib without ICH were, after a successful pilot study,
treated with 0.5 g/kg IVIG starting at 28 wks gestation. The control group consisted of
women with FNAIT treated with 1.0 g/kg, matched for gravidity and platelet count in the
affected sib. Primary outcome variables were occurrence of ICH and platelet count in cord
blood at birth. RESULTS: A total of 62 pregnancies were included, 31 in each group. Fetal
blood sampling was not done in any patient. None of the 62 neonates had ICH on cranial
ultrasound. Mean platelet count in the affected sibs was 16x109/l (range 4-44) in the low
69
dose group and 12x 109/l(range 2-49) in the standard dose group. Mean newborn platelet
counts in the treated patients was 113x 109/l (8-267) in the low dose group versus 110x
109/l (11-279) (p=NS) in the 1 g/kg group, with 1 resp. 2 neonates with a platelet count <
30x 109/l (p=NS). CONCLUSIONS: In pregnancies with FNAIT with a previous affected child
without ICH, IVIG in a weekly dose of 0.5 g/kg maternal weight appears to be as effective
as the commonly used 1 g/kg.
Source: EMBASE
26. Prevention of venous thromboembolism in medical patients with
thrombocytopenia or with platelet dysfunction: A review of the literature
Author(s): Tufano A., Guida A., Dario Di Minno M.N., Prisco D., Cerbone A.M., Di Minno G.
Citation: Seminars in Thrombosis and Hemostasis, 2011, vol./is. 37/3(267-274), 00946176;1098-9064 (2011)
Publication Date: 2011
Abstract: Current guidelines for venous thromboembolism (VTE) primary prophylaxis are
based on randomized clinical trials that exclude subjects at a potentially high bleeding risk.
Thus no specific recommendation/algorithm for pharmacological prophylaxis in patients
with thrombocytopenia and/or platelet dysfunction is available. Because at least 25% of
subjects admitted to medical departments exhibit these conditions, information on this
subject is provided here to optimize their VTE prophylaxis. Low platelet number/function
and clotting abnormalities are common in patients with liver cirrhosis. However, these
patients have a high incidence of portal and idiopathic venous thromboses, implying that
cirrhotic coagulopathy does not protect against thrombosis. At variance with severe
thrombocytopenia (< 50,000/muL), mild/moderate thrombocytopenia (> 50,000/muL)
should not interfere with VTE prevention decisions. In severe thrombocytopenia,
prophylaxis should be considered on an individual basis, however. In patients with
antiphospholipid antibodies and thrombocytopenia, a thrombotic tendency is usually
associated rather than a bleeding risk. VTE prophylaxis in high-risk conditions is thus
suggested in these patients. Except in cases with contraindications to anticoagulation,
antithrombotic prophylaxis should be always considered in hospitalized cancer patients
with thrombocytopenia, especially in those with hematologic malignancies and multiple
VTE risk factors. Aspirin treatment is not as effective as heparins in lowering the risk of
VTE. Studies in stroke suggest that thromboprophylaxis with heparins is safe in patients
with ischemic stroke undergoing aspirin treatment. The need for VTE prophylaxis in
patients on chronic treatment with aspirin and/or clopidogrel should be evaluated after
assessing the individual risk-benefit ratio. Copyright 2011 by Thieme Medical Publishers,
Inc.
Source: EMBASE
27. Rituximab for management of refractory pregnancy-associated immune
thrombocytopenic purpura
Author(s): Gall B., Yee A., Berry B., Birchman D., Hayashi A., Dansereau J., Hart J.
Citation: Journal of obstetrics and gynaecology Canada : JOGC = Journal d'obstetrique et
gynecologie du Canada : JOGC, December 2010, vol./is. 32/12(1167-1171), 1701-2163
(Dec 2010)
Publication Date: December 2010
Abstract: Rituximab is a novel therapy for immune thrombocytopenic purpura (ITP);
however, information about its safety in pregnancy is limited. This case illustrates the
successful use of rituximab to treat pregnancy-associated ITP. A 34-year-old woman
presented with severe ITP at 23 weeks' gestation. Standard treatment with
70
corticosteroids, intravenous immune globulin, and splenectomy failed to raise the platelet
count. Due to ongoing bleeding, rituximab was given in the 26th week of pregnancy. The
platelet count rose to over 100 x 10(9)/L after four weeks. The neonatal B-lymphocyte
count normalized at four months after delivery. There were no neonatal complications of
rituximab therapy. Rituximab may be safe for use in treating pregnancy-associated ITP.
This case highlights the need to investigate further the safety and efficacy of rituximab in
pregnancy.
Source: EMBASE
28. Established venous thromboembolism therapies: Heparin, low molecular weight
heparins, and vitamin K antagonists, with a discussion of heparin-induced
thrombocytopenia
Author(s): Pendleton R.C., Rodgers G.M., Hull R.D.
Citation: Clinics in Chest Medicine, December 2010, vol./is. 31/4(691-706), 0272-5231
(December 2010)
Publication Date: December 2010
Abstract: For a majority of patients with venous thromboembolism (VTE), initial treatment
is straightforward and necessitates the immediate initiation of a parenteral anticoagulant
(eg, heparin or low molecular weight heparin), simultaneous initiation of long-term
therapy (eg, vitamin K antagonist), and discontinuation of the parenteral anticoagulant
after 5 days assuming that the vitamin K antagonist is therapeutic. This standardized
approach is based on numerous pivotal clinical trials completed over the past 3 decades.
Yet, advances in standardized VTE treatment continue to evolve and include issues related
to the selection and dosing of parenteral anticoagulants (eg, relative efficacy and dosing in
the obese patient, patients with renal impairment, and pregnant patients), optimal
location of initial care delivery, use of dosing initiation nomograms for vitamin K
antagonists with the potential of gene-based dosing, and demonstration that longterm
low molecular weight heparin therapy may be optimal for some patient populations (eg,
those with active cancer). Further, in parallel with the evolution of VTE treatment, there
have been remarkable advances in our understanding of heparin-induced
thrombocytopenia, a prothrombotic complication of parenteral anticoagulant use. 2010
Elsevier Inc.
Source: EMBASE
29. Different disparities of gender and race among the thrombotic thrombocytopenic
purpura and hemolytic-uremic syndromes
Author(s): Terrell D.R., Vesely S.K., Hovinga J.A.K., Lammle B., George J.N.
Citation: American Journal of Hematology, November 2010, vol./is. 85/11(844-847), 03618609;1096-8652 (November 2010)
Publication Date: November 2010
Abstract: Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome
(HUS) represent multiple disorders with diverse etiologies. We compared the gender and
race of 335 patients enrolled in the Oklahoma TTP-HUS Registry across 21 years for their
first episode of TTP or HUS to appropriate control groups. The relative frequency of
women and white race among patients with TTP-HUS-associated with a bloody diarrhea
prodrome and the relative frequency of women with quinine-associated TTP-HUS were
significantly greater than their control populations. The relative frequency of women and
black race among patients with idiopathic TTP and TTP-associated with severe ADAMTS13
deficiency was significantly greater than their control populations. The relative frequency
of black race among patients who had systemic lupus erythematosus (SLE) preceding TTP
71
was significantly greater than among a population of patients with SLE, and the relative
frequency of black race among patients with other autoimmune disorders preceding TTP
was significantly greater than their control population. No significant gender or race
disparities were present among patients with hematopoietic stem cell transplantationassociated thrombotic microangiopathy, TTP associated with pregnancy, or TTP associated
with drugs other than quinine. The validity of these observations is supported by the
enrollment of all consecutive patients across 21 years from a defined geographic region,
without selection or referral bias. These observations of different gender and race
disparities among the TTP-HUS syndromes suggest the presence of different risk factors
and may serve as starting points for novel investigations of pathogenesis. 2010 Wiley-Liss,
Inc.
Source: EMBASE
30. How I treat patients with thrombotic thrombocytopenic purpura: 2010
Author(s): George J.N.
Citation: Blood, November 2010, vol./is. 116/20(4060-4069), 0006-4971;1528-0020 (18
Nov 2010)
Publication Date: November 2010
Abstract: Thrombotic thrombocytopenic purpura (TTP) is the common name for adults
with microangiopathic hemolytic anemia, thrombocytopenia, with or without neurologic
or renal abnormalities, and without another etiology; children without renal failure are
also described as TTP. The diagnosis of TTP is an indication for plasma exchange
treatment, but beginning treatment requires sufficient confidence in the diagnosis to
justify the risk of plasma exchange complications. Documentation of a severe deficiency of
plasma ADAMTS13 activity, defined as less than 10% of normal, is not essential for the
diagnosis of TTP. Some patients without severe ADAMTS13 deficiency may benefit from
plasma exchange treatment; in addition, some patients with severe ADAMTS13 deficiency
may subsequently be diagnosed with another cause for their clinical features. However,
severe acquired ADAMTS13 deficiency does define a subgroup of patients who appear to
benefit from treatment with corticosteroids and other immunosuppressive agents in
addition to plasma exchange but who have a high risk for relapse. Approximately 80% of
patients survive their acute episode, a survival rate that has not changed since the
introduction of plasma exchange treatment. Although recovery may appear to be
complete, many patients have persistent minor cognitive abnormalities. More effective as
well as safer treatment for TTP is needed. 2010 by The American Society of Hematology.
Source: EMBASE
Full Text:
Available in fulltext at Highwire Press
31. Successful surgical management of massive pulmonary embolism during the
second trimester in a parturient with heparin-induced thrombocytopenia
Author(s): Hajj-Chahine J., Jayle C., Tomasi J., Corbi P.
Citation: Interactive Cardiovascular and Thoracic Surgery, November 2010, vol./is.
11/5(679-681), 1569-9293 (November 2010)
Publication Date: November 2010
Abstract: Cardiopulmonary bypass during pregnancy is associated with a high fetal and
maternal mortality. We report a successful pulmonary embolectomy in a woman at the
27th week of pregnancy; we performed surgical pulmonary embolectomy under
cardiopulmonary bypass to restore adequate hemodynamic stability and to relieve right
72
ventricle strain. We discuss the decision made for the preferred anticoagulation drug in
the setting of heparin-induced thrombocytopenia in the gravida. The pregnancy was
carried to term and she delivered a healthy boy at 38 weeks of gestation. 2010 Published
by European Association for Cardio-Thoracic Surgery.
Source: EMBASE
Full Text:
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32. Treatment of idiopathic thrombocytopenic purpura in pregnancy with pulsed dose
of dexamethasone
Author(s): Grgic O., Ivanisevic M., Delmis J.
Citation: Journal of Obstetrics and Gynaecology, November 2010, vol./is. 30/8(864), 01443615;1364-6893 (November 2010)
Publication Date: November 2010
Source: EMBASE
Full Text:
Available in fulltext at EBSCO Host
33. Fetal genotyping for platelets antigens: A precise tool for alloimmune
thrombocytopenia: Case report and literature review
Author(s): Nomura M.L., Couto E., Martinelli B.M., Barjas-Castro M.L., Barini R., Passini
Junior R., Castro V.
Citation: Archives of Gynecology and Obstetrics, November 2010, vol./is. 282/5(573-575),
0932-0067 (November 2010)
Publication Date: November 2010
Abstract: Maternal-fetal alloimmune thrombocytopenia complicates about 0.1% of all
pregnancies and is associated with major fetal and neonatal morbidity and mortality,
especially spontaneous central nervous system bleeding leading to death and neurological
handicaps. Successful prevention and treatment depend on the identification of at-risk
possible carriers of anti-platelet antibodies. Case report: We report a case of a mother
with a previous child that developed neonatal hemorrhage; HPA-5b anti-platelet
antibodies were detected post-natally. During the next pregnancy, fetal genotyping
confirmed the presence of HPA-5b antigen; she was treated with weekly intravenous
human immunoglobulin and oral prednisone. Pregnancy evolved without remarkable
features and a full-term baby was delivered, with normal platelet counts. Conclusion:
Fetal alloimmune thrombocytopenia is a potentially lethal condition, but early detection
and prevention lead to successful outcome in most cases. 2010 Springer-Verlag.
Source: EMBASE
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34. A retrospective analysis of obstetric patients with idiopathic thrombocytopenic
purpura: A single center study
Author(s): Fujita A., Sakai R., Matsuura S., Yamamoto W., Ohshima R., Kuwabara H., Okuda
M., Takahashi T., Ishigatsubo Y., Fujisawa S.
Citation: International Journal of Hematology, October 2010, vol./is. 92/3(463-467), 092573
5710 (October 2010)
Publication Date: October 2010
Abstract: Idiopathic thrombocytopenic purpura (ITP) commonly affects women of
childbearing age. We studied the clinical characteristics of pregnant women with ITP to
estimate their risks of bleeding. A retrospective chart review was performed for all
obstetric patients with ITP who had delivery at our hospital, from 1 March 2000 to 31
March 2008. Twenty women with ITP delivered 24 children in 23 pregnancies. In all, eight
women were treated with corticosteroid during their pregnancy period, and there was
only one non-responder. There was no correlation between the maternal platelet count
and the amount of blood loss at delivery. Two infants were revealed to have had platelet
counts lower than 30 x 109/L, and were treated with high-dose IV IgG. One of them also
received corticosteroid therapy. There was no relationship between maternal platelet
count at delivery and infant platelet count at birth. Overall, no serious bleeding event was
seen in either of the mothers or infants. For most women with ITP, pregnancy is
uncomplicated, and even those with severe thrombocytopenia during pregnancy have
good outcomes when under the strict care of a hematologist and gynecologist. 2010 The
Japanese Society of Hematology.
Source: EMBASE
35. A case of multiple thrombotic thrombocytopenic purpura relapses in pregnancy
Author(s): Rommens K., Puhl A., Schinzel H., Klbl H.
Citation: Archives of Gynecology and Obstetrics, October 2010, vol./is. 282/(S77), 09320067 (October 2010)
Publication Date: October 2010
Abstract: Objective: Thrombotic thrombocytopenic purpura (TTP) is a rare but severe
multisystem disorder. It has for a long time been a pathology that was difficult and
frustrating to treat with a infaust prognosis. Since the introduction of plasma
manipulation techniques, particularly plasmaexchange (PE), these patients have benefited
greatly. Materials and methods: In our case report we introduce a 24 year old pregnant
woman whose life probably depended on plasma-exchange techniques several times
during her pregnancy and after the birth of her son. The followup of this woman was
possible by regularly blood counts because she was a clinically stable patient, known with
a history of TTPrelapses. But there is still discussion about the most adequate tests of
diagnosing TTP in pregnant women to differ from hemolysis, elevated liver enzymes and
low platelets syndrome (HELLP). Conclusions: Both pathologies have parallels in clinical
signs and laboratory testings but need a different therapy. The deficiency of ADAMTS-13
activity is suggested to possibly be a prognostic factor of TTP-relapses in pregnancies and
could be the best indicator to start therapy. On the other hand, the costs make it not
attractive as a prognostic test. A literature study shows that, because of the rarity of the
disease there is a need of international cooperative trials to give us more information on
still unanswered questions.
Source: EMBASE
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36. Reconsidering fetal and neonatal alloimmune thrombocytopenia with a focus on
screening and prevention
Author(s): Skogen B., Killie M.K., Kjeldsen-Kragh J., Ahlen M.T., Tiller H., Stuge T.B.,
Husebekk A.
74
Citation: Expert Review of Hematology, October 2010, vol./is. 3/5(559-566), 1747-4086
(October 2010)
Publication Date: October 2010
Abstract: Uncertainty regarding the pathophysiology of fetal and neonatal alloimmune
thrombocytopenia (FNAIT) has hampered the decision regarding how to identify, followup and treat the women and children with this potentially serious condition. Since
knowledge of the condition is derived mainly from retrospective studies, understanding of
the natural history of this condition remains incomplete. General screening programs for
FNAIT have still not been introduced, mainly because of a lack of reliable risk factors and
effective treatment. Now, several prospective screening studies involving up to 100,000
pregnant women have been published and the results have changed the understanding of
the pathophysiology of FNAIT and, thereby, the approach toward diagnostics, prevention
and treatment in a more appropriate way. 2010 Expert Reviews Ltd.
Source: EMBASE
37. Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia:
Systematic review
Author(s): Kamphuis M.M., Paridaans N., Porcelijn L., De Haas M., Van Der Schoot C.E.,
Brand A., Bonsel G.J., Oepkes D.
Citation: BJOG: An International Journal of Obstetrics and Gynaecology, October 2010,
vol./is. 117/11(1335-1343), 1470-0328;1471-0528 (October 2010)
Publication Date: October 2010
Abstract: Please cite this paper as: Kamphuis M, Paridaans N, Porcelijn L, De Haas M, van
der Schoot C, Brand A, Bonsel G, Oepkes D. Screening in pregnancy for fetal or neonatal
alloimmune thrombocytopenia: systematic review. BJOG 2010;117:1335-1343.
Background: Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a potentially
devastating disease, which may lead to intracranial haemorrhage (ICH), with neurological
damage as a consequence. In the absence of screening, FNAIT is only diagnosed after
bleeding symptoms, with preventive options limited to a next pregnancy. Objectives: To
estimate the population incidence of FNAIT and its consequences to prepare for study
design of a screening programme. Search strategy: An electronic literature search using
MEDLINE, EMBASE and Cochrane database, and references of retrieved articles. No
language restrictions were applied. Selection criteria: Prospective studies on screening for
human platelet antigen 1a (HPA-1a) alloimmunisation in low-risk pregnant women. Data
collection and analysis: Two reviewers independently assessed studies for inclusion and
extracted data. Main outcome data were prevalence of HPA-1a negativity, HPA-1a
immunisation, platelet count at birth and perinatal ICH. We aimed to compare outcome
with and without intervention. Main results: HPA-1a alloimmunisation occurred in
294/3028 (9.7%) pregnancies at risk. Severe FNAIT occurred in 71/227 (31%) immunised
pregnancies, with perinatal ICH in 7/71 (10%). True natural history data were not found
because interventions were performed in most screen-positive women. Authors'
conclusions Screening for HPA-1a alloimmunisation detects about two cases in 1000
pregnancies. The calculated risk for perinatal ICH of 10% in pregnancies with severe FNAIT
is an underestimation because studies without interventions were lacking. Screening of all
pregnancies together with effective antenatal treatment such as intravenous
immunoglobulin may reduce the mortality and morbidity associated with FNAIT. RCOG
2010 BJOG An International Journal of Obstetrics and Gynaecology.
Source: EMBASE
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38. Fetal/neonatal alloimmune thrombocytopenia (FNAIT): The importance of an
accurate diagnosis for future pregnancies
Author(s): Canals C., Ibanez M., Gracia M., Farssac E., Vinyets I., Tarrago M., Nogues N.,
Muniz-Diaz E.
Citation: Vox Sanguinis, October 2010, vol./is. 99/(22), 0042-9007 (October 2010)
Publication Date: October 2010
Abstract: Background: We present the first FNAIT case reported in Spain due to an antiHPA-2b allo-immunization. Case Report: A 35-year old healthy woman gave birth to her
first child in the 40th week by caesarean section, for obstetric indication. No diathesis was
observed in the newborn. The platelet count was: 38 x 109/L. No infections or other
causes of early- onset thrombocytopenia were present. Methods: Platelet auto-antibodies
in the mother's serum were excluded by immunofluorescent tests (IF). No HLA antibodies
were detectable (ELISA Quick Screening). The solid phase assay (PAK12) test did not reveal
alloantibodies against IIb-IIIa glycoprotein (GP), Ib-IX GP or Ia-IIa GP. The MAIPA assay
allowed us to identify an anti-HPA-2b alloantibody. This finding was consistent with the
platelet genotype (PCR-SSP). Results: Anti-HPA-3b or HPA-15b antibodies were excluded
by IF and MAIPA assays. The mother was initially typed as HPA 1b1b by PCR-SSP. Further
studies showed that she was a carrier of the polymorphism 262T>C, which may lead to
false HPA-1a negative results by PCR-SSP, and she was actually 1a1b. Conclusion:
Antibodies were only detectable by MAIPA using a GPIb/IX monoclonal antibody. This fact,
together with the polymorphism found in the mother, stresses the need to perform the
appropriate laboratory investigations to identify the antibodies implicated in FNAIT cases.
An accurate diagnosis is needed in order to ensure a good clinical management in
subsequent pregnancies.
Source: EMBASE
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39. Clinical outcomes of intrauterine platelet transfusions in fetomaternal
alloimmune thrombocytopenia (FMAIT) in England
Author(s): Lucas G., Calvert A., Green F., Ranasinghe E., Roberts D., Sadani D., Green A.
Citation: Vox Sanguinis, October 2010, vol./is. 99/(19), 0042-9007 (October 2010)
Publication Date: October 2010
Abstract: Background: Management of pregnancies at risk of FMAIT varies according to
the previous history and between centres. There is a trend away from intrauterine platelet
transfusions (IUT) because of the associated risks and towards maternal IVIg therapy but
fetal blood sampling (FBS) continues to be used to monitor responses to IVIg with IUTs
being given to thrombo- cytopenic fetuses. Methods: Platelets for IUT were from
accredited HPA-1a(-)5b(-) donors, lacking red cell, HLA and HPA antibodies. Treatment
outcomes were followed by contacting the respective centres. Results: FBS was
performed for 42 pregnancies at risk of severe FMAIT due to HPA-1a (36), 5b (3), 1a+5b
(2), or 3a (1) antibodies over a 2 year period. Weekly IVIg and IUTs were used to treat 23
pregnancies and in a further three pregnancies steroids were also used. Five pregnancies
did not receive IUTs because the fetal platelet counts were satisfactory and 11
76
pregnancies were treated with IUTs but other treatments were incompletely documented.
A total of 137 IUTs were given (Range: 0-14 per pregnancy; Mean: 3.7). The mean platelet
increment was 429 (range 71-881) for 100 evaluable IUTs. Seven neonates required
further platelet transfusions after birth. There were three deaths following IUT, two
emergency deliveries for bradycardia after IUT and one intracardiac transfusion following
a hepatic vein bleed. Intracranial haemorrhages were not detected in the neonates.
Conclusion: IUTs continue to be used in cases of FMAIT refractory to IVIg therapy. The
procedure had a combined morbidity/ mortality of 14.3% in this study.
Source: EMBASE
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40. The usefulness of reticulated platelet count for determining the type of
thrombocytopenia in pregnant women
Author(s): Uhrynowska M., Maslanka K., Kopec I., Sakiewicz J., Lopacz P., Orzinska A.,
Brojer E.
Citation: Vox Sanguinis, October 2010, vol./is. 99/(17), 0042-9007 (October 2010)
Publication Date: October 2010
Abstract: Background: Reticulated platelets (RP) are the youngest platelets (PLT) released
from megacaryocytes. Investigation of RP percentage is useful in patients with plateletconsumptive disorders, such as idiopathic thrombocytopenic purpura (ITP). Thrombocytopenia (Tcp) in pregnancy affects 6-24% of all pregnancies; ~75% of these cases are
diagnosed as gestational thromocytopenia (GT). During pregnancy it is difficult to
distinguish GT from ITP and sometimes from congenital thrombocytopenia (CT). The aim
of our study was to assess the usefulness of RP count in determining the type of Tcp in
pregnant women. Methods: Pregnant women: 22 healthy (Control); 49 with Tcp - platelet
count <100 x 109/L (21 of which had GT, 22 - ITP and 6 - CT). All were under the medical
care of the Institute of Hematology & Transfusion Medicine. METHODS The RP percentage
was determined using flow cytometric analysis (PE-labeled MoAb CD41 and thiazole
orange) and expressed as mean +/- SD (Newman- Keuls correlation test). Results: The RP
level in women with ITP and CT was higher than the RP count in women with GT and in
control group. Conclusion: The differences in the RP level in ITP and CT pregnant women
in comparison with the RP in GT and control were not statistically significant, probably due
to the insufficient number of women tested. The final conclusions could be drawn on
larger study group.
Source: EMBASE
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41. Subdural haematoma in pregnancy-induced idiopathic thrombocytopenia:
Conservative management
Author(s): Pandey M., Saraswat N., Vajifdar H., Chaudhary L.
Citation: Indian Journal of Anaesthesia, September 2010, vol./is. 54/5(470-471), 00195049 (September - 2010)
Publication Date: September 2010
Abstract: Conservative management of subdural haematoma with antioedema measures
in second gravida with idiopathic thrombocytopenic purpura (ITP) resulted in resolution of
haematoma. We present a case of second gravida with ITP who developed subdural
77
haematoma following normal vaginal delivery. She was put on mechanical ventilation and
managed conservatively with platelet transfusion, Mannitol 1g/kg, Dexamethasone
1mg/kg and Glycerol 10ml TDS. She regained consciousness and was extubated after 48
hrs. Repeat CT after 10 days showed no mass effect with resolving haematoma which
resolved completely after 15 days. Trial of conservative management is safe in pregnant
patient with ITP who develops subdural haematoma.
Source: EMBASE
Full Text:
Available in fulltext at National Library of Medicine
42. Automated plateletpheresis is safe in second and third trimester pregnant women
for the treatment of Fetal Alloimmune Thrombocytopenia (FAIT)
Author(s): Walsh R.P., Arinsburg S.A., Pham H.P., Grima K.M.
Citation: Transfusion, September 2010, vol./is. 50/(87A-88A), 0041-1132 (September
2010)
Publication Date: September 2010
Abstract: Background: FAIT results from the destruction of fetal platelets (PLTs) by
transplacental maternal antibodies (Abs) reactive against fetal antigens (Ags) inherited
from the father. Approximately 50% of cases occur during the first pregnancy and disease
severity increases in 80-90% of subsequent pregnancies. Most cases are asymptomatic but
intracranial hemorrhage has been reported in 7-26% of cases with up to a 30% mortality
rate. Incompatibility in the human PLT Ag system (HPA) is the most common cause,
usually the result of maternal anti-HPA-1a Abs against HPA-1a Ags on fetal PLTs, but Abs
against other PLT or human leukocyte Ags can lead to FAIT. Using washed maternal PLTs
for intrauterine PLT transfusion (IUPT) ensures compatibility as the specific causative
antibody is not always known. We sought to determine the safety of automated PLT
apheresis for women during the second or third trimester of pregnancy. Methods: A
retrospective review of all PLT apheresis procedures on pregnant women at our institution
from 2/2003 to 11/2008 was performed. An order for PLT apheresis and medical approval
was obtained from the donors obstetrician 1 week prior to collection. Donors were reevaluated one day prior to collection. A nonstress test was performed after the procedure
to ensure fetal health. All available data including predonation PLT count and hemoglobin
(Hb), age, gestational age, PLT yield, RBC and plasma loss, length of collection, and
adverse reactions were recorded. Donors were required to meet all donor criteria
excluding deferral for pregnancy and Hb <11 g/dL. Donors with Hb <11 g/dL on the ABX
Micro60 required physician approval. All PLT apheresis procedures were performed on a
Trima Accel 5.1 or 4.0 according to our standard operating procedure with an
extracorporeal volume (ECV) of approximately 200 mL. Results: 27 autologous collections
were performed on 22 females (age 33.4 +/- 4.7 years) at 30.6 +/- 4.6 weeks gestation
with a mean Hb of 10.8 +/-1.4 g/dL, range 8.5-12.7 g/dL. 14 collections were performed
with a Hb <11 g/dL. All donors had a PLT count >150K/L (mean PLT count 232.3 +/51.5K/L). No adverse events or significant changes in vital signs were noted during or
immediately after all procedures. One donor requested early termination for unknown
reasons; however, the yield was 3.63x1011PLTs. We were not notified of any post
donation reactions. Mean collection times were 69 +/- 18 minutes. The mean plasma and
red cell losses were 325.7 +/- 66.4 mL and 43.5 +/- 0.6 mL, respectively. All but one of the
collections (yield 2.1x1011 PLTs) met the minimum criteria for PLT yield (mean yield 4.2 +/1.1x 1011 PLTs). Conclusion: Our results indicate that PLT apheresis is a safe procedure for
women in the second and third trimester of pregnancy for the treatment of FAIT. The
well-tolerated ECV despite low Hb is likely secondary to the expanded plasma volume
78
seen in pregnancy.
Source: EMBASE
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43. Animal model of fetal and neonatal immune thrombocytopenia: Role of neonatal
Fc receptor in the pathogenesis and therapy
Author(s): Chen P., Li C., Lang S., Zhu G., Spring C., Freedman J., Ni H.
Citation: Transfusion, September 2010, vol./is. 50/(6A-7A), 0041-1132 (September 2010)
Publication Date: September 2010
Abstract: Background: Fetal and neonatal alloimmune thrombocytopenia (FNIT) is a lifethreatening bleeding disorder in which maternal antibodies cross the placenta and
destroy fetal platelets, targeting the platelet glycoprotein GPIIIa (3 integrin). The neonatal
Fc receptor (FcRn) regulates IgG homeostasis and plays an important role in maternal IgG
placental transportation. However, the role of FcRn in FNIT has not been adequately
studied. Methods: Here, we generated a new strain of mice with combined deficiencies of
3 integrin (3) and FcRn (FcRn). Results: 3FcRnmice mount an immune response and
generate anti-3 integrin antibodies following immunization with 3/FcRnplatelets. Different
isotypes of IgGs (both Th1- and Th2-like immune responses) were generated, which
induced thrombocytopenia. To establish a mouse model of FNIT, we bred 3/FcRnmales
with immunized 3FcRnfemale mice (immunized twice with 3/FcRnplatelets), and nave
3/FcRnfemale mice bred with 3/FcRnmales as controls. Anti-mouse 3 integrin IgG was
detected in these FcRn-negative females (3FcRn-) both during pregnancy and after
delivery. However, postpartum platelet counts in 3pups (3FcRn-) were not decreased
compared to controls. FNIT and anti-3 integrin IgG were not observed in these pups.
Nevertheless, platelet counts were dramatically decreased in pups (3FcRn-/-) delivered
from immunized, FcRn-positive mice (3FcRn/mothers bred with 3FcRn/males; both
circulating and platelet-associated anti-3 integrin IgG were detected in pups. To
distinguish whether maternal or fetal FcRn contributes to FNIT, we bred immunized
female (3FcRnand 3FcRn) mice with male (3/FcRnand 3/FcRn) mice to generate 3FcRnand
3FcRnpups. FNIT was only detected in the 3FcRnpups but not in 3FcRnpups, yet they came
from the same mother. Thus, fetal (not maternal) FcRn was required for transplacental
IgG transportation and FNIT pathology. To test the therapeutic efficacy of anti-FcRn
monoclonal antibody in FNIT, anti-FcRn (5 mg/kg) was injected into the immunized
pregnant 3FcRn/mice. Anti-FcRn downregulated anti-platelet IgG in both the maternal and
fetal circulations, and ameliorated FNIT. We also found IVIG (1g/kg/week) administration
decreased the anti-3 integrin IgG in pregnant 3FcRn/and 3FcRnmice, suggesting that IVIG
downregulates pathogenic IgG via both FcRn-dependent and independent pathways.
Conclusion: FcRn is required for placental transportation of all isotypes of IgGs and is
essential for pathogenesis of FNIT. Fetal, but not maternal, FcRn is required for these
processes and anti-FcRn may be an efficient therapy for FNIT. Our data also demonstrate
that IVIG downregulated maternal pathogenic antibody via both FcRn-dependent and
independent pathways.
Source: EMBASE
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44. Labor analgesia in patient with immune thrombocytopenic purpura
Author(s): Schuitemaker Requena J.B., Pantaleon L.L.A., Rodriguez Perez C.L., Tejada Perez
P., De Armas Marrn N., Emperador F.
Citation: Regional Anesthesia and Pain Medicine, September 2010, vol./is. 35/5(E171),
1098-7339 (September-October 2010)
Publication Date: September 2010
Abstract: Introduction: Thrombocytopenia occurs in approximately 10% of all
pregnancies1. ITP is responsible of 5%, with an incidence of 1:1000 gestations2 We present
a case of a patient with Immune thrombocytopenic purpura (ITP) during pregnancy. Case
Report: A 34 years old, second gestation, who debuted for ITP in her first pregnancy, she
was complicated with postpartum hemorrhage due to genital laceration by instrumental
delivery. She merited the transfusion of 2 red blood cells packages. The newborn
developed transitory thrombocy-topenia and the thrombocytopenia reverted during the
puerperium. The laboratory findings at day of admission were: Hb 13 g/dL, Hto 38.5%,
platelets 81.000/|xl, PT 13.3 seg, PTT 27.9 seg, INR 1.0, Quick index 107%. She was in good
general conditions. She requested analgesia at 8 cm of cervical dilation and we proposed
epidural analgesia. With the patient in sit position we perfom an epidural analgesia
without complications, trougth epidural catheter was administered 10 mL of 0.125%
bupivacaine plus 100 micrograms of fentanyl. Twenty five min after the first dose and with
complete cervical dilation, a new dose of 5 mL of 0.2% plain bupivacaine was collocated. A
newborn was obtained with weight of 3150 g and height 52 cm, Apgar: 9, 10 y 10 points at
10,50 and 100 minute respectively. The fetal arterial blood gasses were pH 7.176, EB:-4.4.
During her stay at post anesthetic room, she did not present any eventuality. Platelets
counts were 87000/mm3 and the epidural catheter was removed. In conclusion, seems
safe the realization of neuroaxial blocks with platelets values highest of 50000/mm3, and
we considered a must done request platelets count before the epidural catheter removal.
Source: EMBASE
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45. Successful prevention of thrombotic thrombocytopenic purpura (TTP) relapse
using monthly prophylactic plasma exchanges throughout pregnancy in a patient with
systemic lupus erythematosus and a prior history of refractory TTP and recurrent fetal loss
Author(s): Abou-Nassar K., Karsh J., Giulivi A., Allan D.
Citation: Transfusion and Apheresis Science, August 2010, vol./is. 43/1(29-31), 1473-0502
(August 2010)
Publication Date: August 2010
Abstract: Background: The occurrence of thrombotic thrombocytopenic purpura (TTP) in
the setting systemic lupus erythematosus (SLE) is rare. In women of childbearing age, TTP
is associated with high rates of recurrence in pregnancy. Furthermore, both TTP and SLE
are associated with a significant risk of adverse pregnancy outcomes. Case presentation:
We describe the case of a 36. year old female in her first trimester of pregnancy with a
prior history of SLE-associated severe refractory TTP who was treated with a combination
of corticosteroids and prophylactic plasma exchanges (PLEX) throughout pregnancy to
prevent TTP recurrence. She delivered a healthy infant at 33. weeks of gestation after the
80
onset of preterm labor. There was no evidence of TTP recurrence in the antepartum or
postpartum period in this high risk patient. Conclusion: Prophylactic PLEX should be
considered as a therapeutic option to prevent recurrent TTP during pregnancy in high risk
patients, including patients with previous SLE-associated TTP. 2010 Elsevier Ltd.
Source: EMBASE
46. Successful pregnancy in a case of congenital thrombotic thrombocytopenic
purpura
Author(s): Meti S., Paneesha S., Patni S.
Citation: Journal of Obstetrics and Gynaecology, July 2010, vol./is. 30/5(519-521), 01443615;1364-6893 (July 2010)
Publication Date: July 2010
Source: EMBASE
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47. A case of pregnancy-induced thrombotic thrombocytopenic purpura with a kidney
allograft recipient
Author(s): Iwami D., Harada H., Hotta K., Miura M., Seki T., Togashi M., Hirano T.
Citation: Clinical Transplantation, July 2010, vol./is. 24/SUPPL. 22(66-69), 0902-0063;13990012 (July 2010)
Publication Date: July 2010
Abstract: A 32-yr-old female patient, who had been suffering from diffuse crescentic
glomerulonephritis and a consequent end-stage renal disease, successfully underwent
living-related ABO-incompatible kidney transplantation after a desensitization therapy
including anti-CD20 monoclonal antibody. Forty-six months after the transplantation, the
recipient became pregnant. At the 17th gestational week, the patient was admitted for
the management of pregnancy-induced hypertension and aggressive deterioration of
kidney graft function. At the 21st gestational week, the patient lost her kidney graft and
was re-induced into regular hemodialysis. The patient was also suffering from progressive
hemolytic anemia, thrombocytopenia, and neurologic symptoms with decreased activity
of von Willebrand factor-cleaving protease, a disintegrin-like and metalloprotease with
thrombospondin type 1 motifs 13 (ADAMTS13). From these findings and a kidney allograft
biopsy, the patient was diagnosed as thrombotic thrombocytopenic purpura concurrent
with acute T-cell-mediated rejection. The patient immediately underwent plasma
exchange as well as steroid pulse therapy. Despite these treatments, thrombocytopenia
and intrauterine growth retardation progressed. The patient underwent a caesarian
section at the 24th gestational week. Consequently, her platelet count recovered
drastically. However, the patient lost her neonate five d after giving a birth, and the
patient's graft function had never recovered. 2010 John Wiley & Sons A/S.
Source: EMBASE
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48. Postpartum plasma exchange in a woman with suspected thrombotic
thrombocytopenic purpura (TTP) vs. hemolysis, elevated liver enzymes, and low platelet
syndrome (HELLP): a case study
81
Author(s): Myers L.
Citation: Nephrology nursing journal : journal of the American Nephrology Nurses'
Association, July 2010, vol./is. 37/4(399-402), 1526-744X (2010 Jul-Aug)
Publication Date: July 2010
Abstract: The occurrence of a hypercoagulable state and decreasing concentration of
ADAMTS 13 in late pregnancy and during the postpartum period increases the risk for a
woman to develop life-threatening thrombotic thrombocytopenic purpura (TTP). This is
also the time of great risk for the more common obstetric complications of preeclampsia;
eclampsia; and hemolysis, elevated liver functions tests, low platelets (HELLP) syndrome.
These conditions are associated with high maternal and perinatal mortality. Differential
diagnosis may be difficult due to the overlapping of clinical and laboratory findings,
including thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms,
and renal insufficiency, making it difficult or impossible to distinguish them from TTP.
Management of microangiopathic disorders encountered during pregnancy differ;
therefore, an accurate diagnosis is required. Outcomes of TTP without plasma exchange
therapy (TPE) are almost uniformly fatal. Early recognition and management of symptoms
with prompt and aggressive TPE is essential when TTP is suspected.
Source: EMBASE
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49. Fetal and neonatal alloimmune thrombocytopenia
Author(s): Kaplan-Gouet C.
Citation: Vox Sanguinis, July 2010, vol./is. 99/(2), 0042-9007 (July 2010)
Publication Date: July 2010
Abstract: The fetal and neonatal alloimmune thrombocytopenia results from maternal
immunization against a specific platelet alloantigen paternally inherited by the foetus. This
syndrome is the platelet counterpart of haemolytic disease of the neonate, although it
frequently affects the first infant. The incidence of NAIT has been estimated in unselected
Caucasian population to be 1/800-1/1000 live births by prospective studies. The most
feared complication is intracranial hemorrhage (ICH) in the setting of severe
thrombocytopenia. The morbidity has been estimated to be 20% of the reported cases
and mortality up to 15%. Since the first description of these conditions in the 1950's by
Harrington, significant progress has been made in the laboratory diagnosis and
management of this condition. During pregnancy, fetal thrombocytopenia should be
suspected in a variety of circumstances: recurrent miscarriages, or ICH which may be
diagnosed by sonography. In the neonate, alloimmune thrombocytopenia (NAIT) is the
commonest cause of early onset isolated thrombocytopenia in an otherwise healthy
newborn. NAIT is usually discovered incidentally when a full-term neonate born to a first
time pregnant healthy mother has petechiae, purpura or, less frequently, overt visceral
bleeding at birth or a few hours afterwards. ICH may be present at birth or can occur as
long as the newborn is severely thrombocytopenic. The diagnosis of NAIT is suspected
when other causes of thrombocytopenia are excluded. However, the infant may be
asymptomatic, with thrombocytopenia discovered incidentally. Therefore, unexpected or
unexplained neonatal thrombocytopenia or early onset of severe thrombocytopenia in
both pre-term and term babies should raise the possibility of NAIT and guide
investigations accordingly. The risk of life-threatening hemorrhage necessitates prompt
diagnosis and effective therapy. The laboratory diagnosis is of utmost importance for the
management of the affected infant and the subsequent pregnancies. The diagnosis is
straightforward when a maternal alloantibody is detected directed against the offending
82
antigen present in the infant. The molecular basis of the platelet alloantigens has been
elucidated and a number of genotyping methods have been developed: PCR-RFLP, PCRSSP, real time PCR and more recently microarrays. Although genotyping is widely used,
unknown genetic variants may alter the results, and ethnic diversity is of importance. The
detection of the alloantibodies could be challenging. Currently the most widely techniques
used are the antigen-capture assays with mouse monoclonal antibodies (MAIPA
technique). Alloantibody heterogeneity should be taken into consideration. Detection of
alloantibodies directed against low frequency antigens is somehow more complicated.
New techniques are developed to overcome these problems. When the diagnosis is
equivocal, retesting is recommended with new samples. Difficulties in laboratory
diagnosis should not delay therapy when there is bleeding tendency or severe
thrombocytopenia. Severe neonatal thrombocytopenia necessitates platelet transfusions.
Antenatal management has been developed due to the high rate of recurrence for
subsequent incompatible fetuses, with usually a more severe condition. Maternal therapy
with weekly IVIG with or without corticosteroids and reduction of invasive procedures are
considered as first line approach.
Source: EMBASE
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50. Evaluation of antenatal thrombocytopenia in 115 women attending a tertiary
centre, together with neonatal platelet counts: Can this information help in antenatal
counselling
Author(s): Rajeswary J., Myers B.
Citation: Archives of Disease in Childhood: Fetal and Neonatal Edition, June 2010, vol./is.
95/(Fa57), 1359-2998 (June 2010)
Publication Date: June 2010
Abstract: The aim of this study was to evaluate the maternal platelet count in women
diagnosed antenatally with thrombocytopenia (platelet count < 100) from any cause,
neonatal outcome, and, where available, the corresponding neonatal platelet count. The
authors wanted the information in our population to guide us in the following: (1) for
antenatal counselling, as most women diagnosed with thrombocytopenia are anxious
regarding foetal and neonatal outcomes, (2) Guidance in managing those babies who did
not have a cord blood count done at birth. 115 women with thrombocytopenia were
identified from laboratory records over a 3-year period from a tertiary care unit in a
combined Obstetric haematology clinic. Of the 115 maternities, neonatal platelet counts
were available only on 44 babies (38.2%), of which there were only 2 babies with a count
of less than 100 (4 %) was noted Of the 71 babies who did not have a count one was
stillbirth, due to severe preeclampsia, IUGR and prematurity, none had any notable
neonatal problems. This information is helpful in antenatal counselling of women who are
found to have thrombocytopenia in pregnancy. It will help reduce maternal anxiety
regarding neonatal outcomes. Also these data encourage us to look at larger numbers and
decide if the authors need to do neonatal counts as a routine if a cord blood sample has
been missed.
Source: EMBASE
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51. [Clinical analysis of pregnancy complicated with severe thrombocytopenia]
83
Author(s): Wang D.P., Liang M.Y., Wang S.M.
Citation: Zhonghua fu chan ke za zhi, June 2010, vol./is. 45/6(401-405), 0529-567X (Jun
2010)
Publication Date: June 2010
Abstract: To investigate the etiology and perinatal outcome of pregnancies complicated
with extremely severe thrombocytopenia [at least two times of platelets count (PLT) < 10
x 10(9)/L during pregnancy]. Clinical data, including basic information, etiology,
management and outcomes of pregnant women with extremely severe
thrombocytopenia, admitted to Peking University People's Hospital from January 2004 to
March 2009, were retrospectively collected. The management of these cases varied
according to different etiology and the symptoms: (1) PLT were maintained > 20 x 10(9)/L
and hemoglobulin > 70 g/L in those women without spontaneous bleeding; (2) PLT
transfusion would be required when PLT < 10 x 10(9)/L or bleeding occur and RBC would
be supplied when hematocrit < 25% and hemoglobulin < 70 g/L; (3) Hemoglobulin should
be > 70 g/L and PLT > 30 x 10(9)/L before cesarean section or delivery; (4) Predinisone
and/or intravenous immunoglobulin G (IVIG) would be given in women complicated with
idiopathic thrombocytopenic purpura (ITP) when PLT < (20 - 30) x 10(9)/L or bleeding. PLT
would be given if all the above management were failed, or PLT < 10 x 10(9)/L, or
bleeding. Women without bleeding would be closely monitored and delivery would be
planned. (1) Twenty-six cases were identified among 9302 deliveries during the study
period (0.28%), with an average of maternal age of 29. Seventeen were diagnosed before
conception and 9 during pregnancy. Among the 26 women, half received regular prenatal
check in our hospital and the average gestations at diagnosis was 24 weeks and the other
half without regular prenatal visits and the average gestations at diagnosis was 32 weeks.
Etiology was identified in 24 out of the 26 women, including 14 (54%) ITP, 5
myelodysplastic syndrome (MDS), 4 chronic aplastic anaemia (CAA) and 1 systemic lupus
erythematosus (SLE). (2) Management: All of the 26 women received blood products.
Among the 14 ITP cases, 6 received predinisone and IVIG and 8 only took predinisone.
Nine of the 26 patients (35%) had pregnant complications, among which 6 (6/9) were
preeclampsia. The overall average gestation at delivery was 36 weeks. Only 2 delivered
vaginally with the average blood loss of 83 ml and 23 cesarean sections were performed
with the average blood loss of 410 ml. (3) Perinatal outcomes: There were 26 perinatal
babies, among which 1 died intrauterine and 25 were born alive (12 preterm infants). The
average birth weight was 2877 g. Neonatal severe thrombocytopenia presented in 2
newborns whose mother complicated with ITP. The main cause of extremely severe
thrombocytopenia during pregnancy is ITP, managed mainly by predinisone and IVIG,
followed by CAA and MDS, which may require supportive treatment. Pregnancy
complicated with extremely severe thrombocytopenia is not an indication of termination.
Better maternal and fetal outcomes can be achieved through proper treatment based on
the etiology, intensive care in prevention and management of complications and cesarean
section.
Source: EMBASE
52. Preimplantation genetic diagnosis as a strategy to prevent a fetomaternal
incompatibility for a highly immunogenic platelet antigen causing severe fetal/neonatal
alloimmune thrombocytopenia (FNAIT)
Author(s): Freixa L., Nogues N., Martnez-Pasarell O., Lpez O., Canals C., Bassas L., MuizDiaz E.
Citation: Reproductive BioMedicine Online, May 2010, vol./is. 20/(S15-S16), 1472-6483
(May 2010)
84
Publication Date: May 2010
Abstract: FNAIT is an immunological complication of the gestation caused by maternal IgG
antibodies that recognize fetal platelet antigens inherited from the father.
Alloimmunization against the HPA-1a antigen is the cause of aprox. 85% of the FNAIT
cases in caucasians. Women at risk are only identified after a previous child with FNAIT
and the antenatal treatment of alloimmunized women in subsequent pregnancies is
controversial and not always effective. Aim: To set up and validate a preimplantation
genetic diagnosis protocol based on genotyping the HPA-1a/1b polymorphism from single
blastomeres in order to avoid the fetomaternal HPA-1a incompatibility. Materials and
Methods: Single blastomeres from previously HPA-1 typed couples were obtained by
embryo biopsy. The corresponding genomic DNA was amplified by a modified Multiple
Displacement Amplification procedure using components of the GenomiPhi v.2 kit.
Subsequent analysis of the blastomere's HPA-1 genotype was carried out by Real-time
PCR with allele-specific TaqMan MGB probes. In parallel, a set of previously selected
microsatellite markers were amplified using labeled primers and the allelic profile was
determined by capillary electrophoresis. A total of 14 couples participated in the
validation study. After obtaining informed consent of these couples, whole embryos not
selected for transfer were collected. A total of 70 blastomeres, corresponding to 36
embryos, were tested during this evaluation. Results: We have selected a set of
microsatellite markers adjacent to the HPA-1 locus and have assessed their informativity
in a study including 10 familial FTNAI cases with previously known HPA-1a incompatibility.
A PGD protocol for the detection of HPA-1a negative embryos has been set up, which
includes the analysis of 3 extragenic (D17S1183, D17S806, D17S1827) and 1 intragenic STR
markers. This protocol has been tested in a validation study with single blastomeres (n =
70). Preliminary data analysis indicates an ADO rate of aprox. 15% but we have been able
to identify all HPA-1a false negative embryos with the STRs genetic linkage. As an attempt
to reduce the incidence of ADO, a modification of the current protocol consisting of the
addition of locus-specific primers to the reaction mix during the MDA step is currently
evaluated. Conclusions: This PGD approach offers new prospects to HPA-1a alloimmunized
women with a heterozygous husband and a previous history of an affected NAIT child. The
protocol here described allows to avoid the HPA-1a incompatibility through the selection
of the embryo(s) to be transfered.
Source: EMBASE
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53. Clinical study on five cases of thrombotic thrombocytopenic purpura complicating
pregnancy
Author(s): He Y., Chen Y., Zhao Y., Zhang Y., Yang W.
Citation: International Journal of Laboratory Hematology, May 2010, vol./is. 32/(172-173),
1751-5521 (May 2010)
Publication Date: May 2010
Abstract: Objectives: Thrombotic thrombocytopenic purpura (TTP) is a rare lifethreatening disorder characterized by microangiopathic haemolytic anaemic and
thrombocytopenia. TTP occurs occasionally late in pregnancy or immediately after
delivery. To investigate the early recognition and management of pregnancy in women
with thrombotic thrombocytopenic purpura (TTP) using emergency plasma exchange.
Methods: Five cases of TTP were evaluated retrospectively. Clinical and laboratory
findings, von Willebrand factor (vWF)- cleaving metalloprotease (ADAMTS13) activity, and
maternal and neonatal outcome were recorded and analyzed. Results: Five cases were all
85
nulliparous. ADAMTS13 assay was performed, and the enzyme activity was less than 5% of
the normal controls in three cases. Gene mutation in the 9th exon causing amino acid
exchange 349Arg->Cys in ADAMTS13 was identified in one patient. After treatment
including infusion of fresh-frozen plasma (n=4), packed red blood cells (n=5), platelet
transfusions (n=2) and /or continued renal replacement therapy (CRRT) (n=1), plasma
exchange (n=2), three patients were alive, one died on postpartum day 6 in hospital
without plasma exchange, and one of familial TTP died 3 months after dischange.
Conclusions: TTP complicating pregnancy is rare and associated with high maternal
mortality. The sign of thrombocytopenic and microangiopathic hemolytic anemia before
and after delivery, especially patients with HELLP syndrome highly suggest the diagnosis.
Improved survival after this disorder has been attributed to aggressive treatment with
plasma transfusion or plasmapheresis.
Source: EMBASE
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54. Thrombocytopenia in plasmodium parasitized pregnant women in the niger delta
of Nigeria
Author(s): Osaro E., Zachaeus J., Charles A., Miebaka H.
Citation: International Journal of Laboratory Hematology, May 2010, vol./is. 32/(155-156),
1751-5521 (May 2010)
Publication Date: May 2010
Abstract: Objectives: Malaria infection during pregnancy is a major public health problem
in tropical and subtropical regions of the world. Haematological changes associated with
malaria in pregnancy are not well documented, and have focused predominantly on
anaemia. The aim of this study was to determine the impact of Plasmodium parasitaemia
on the platelet count of pregnant women in the Niger Delta of Nigeria. Methods: : In this
observational study we reviewed the platelet counts from routine complete blood count
(CBC) in a cohort of healthy (pregnant and nonpregnant) and malaria-infected pregnant
women attending antenatal clinics. A platelet count of 100 x 109/L was the threshold at
two standard deviations below the mean for healthy Nigerian pregnant women used to
indicate thrombocytopenia. Differences in platelet counts were compared based on
malaria species and parasitemia in matched nonpregnant and pregnant women. Blood
smears from Quantitative Buffy Coat malaria-positive samples stained with Giemsa were
used for determination of parasite load and specie identification by light microscopy.
Results: The mean platelet counts (x109/L) were significantly lower in pregnant subjects
with an episode of Plasmodium falciparum malaria 111.3 +/- 9.3 x 109/L compared to
nonparasitized and healthy nonpregnant controls (255.09 +/- 24.10 and 270 +/- 51.5 x
109/L) respectively. Platelet count values were 112.5 +/- 9.68 x 109/L and 126.3 +/16.7x109/L for the primigravidae and multigravidae respectively. (chi2= 10.46; P = 0.05).
Parasite density was significantly higher among Plasmodium parasitized primigravidae
compared to multigravidae 2150 (1638-2662) parasites/muL in primigravidae women
compared to 1826 (1430-2222) parasites/muL in multigravid women. The mean parasite
count in Plasmodium falciparum parasitized subjects was 2650 +/- 234 parasites/muL,
95% confidence interval (2092-3118). Malaria parasite was found to exert a significant
reduction in platelet density in parasitized subjects. This reduction was more pronounced
in primigravidae and multigravidae. An inverse relationship was established between
parasite density and platelet count (y = - 0.020 x + 86.2, r = -0.3). Conclusions: There is
need for a strengthened antenatal care system with increased awareness of the problem
among communities most affected by malaria .Preventative strategies including regular
chemoprophylaxis, intermittent preventative treatment with antimalarials and provision
86
of insecticide-treated bed nets should be implemented as well as integration of malaria
control tools with other health programmes targeted to pregnant women and newborns.
Source: EMBASE
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55. Fetomaternal alloimmune thrombocytopenia (FMAIT) in the UK: A prospective
national study of incidence and outcomes using obstetric, paediatric and laboratory
reporting systems
Author(s): Allen D., Knight M., Pierce M., Spark P., Roberts D.J., Murphy M.F.
Citation: British Journal of Haematology, May 2010, vol./is. 149/(11), 0007-1048 (May
2010)
Publication Date: May 2010
Abstract: FMAIT is the commonest cause of severe neonatal thrombocytopenia in
otherwise well term infants and can lead to serious bleeding, intracranial haemorrhage
and death. There is current debate about the efficacy of antenatal screening for the
condition. The aim of this study was to address the deficiency in basic epidemiological
data on FMAIT in order to inform this debate. Parallel national descriptive studies were
conducted using the UK Obstetric Surveillance System (UKOSS) and the British Paediatric
Surveillance Unit (BPSU) for a two year period from October 2006. Cases were crosschecked with the NHS Blood and Transplant laboratories. Data from the three sources
were matched and the overall incidence was estimated using capture-recapture
techniques. One year follow-up data was sought through the BPSU. There were 175 cases
of FMAIT identified through the three sources over a period of two years. After capture
recapture analysis, 196 cases were estimated to have occurred (95%CI 185-208) in
1,332,642 total births, giving an estimated incidence of 1.3 cases per 10,000 births (95%CI
1.1-1.5). There were 9 known pregnancy losses/deaths amongst the 175 cases (one
miscarriage, 3 terminations, 3 stillbirths and two infant deaths). An additional 20 (14%)
infants had an intracranial haemorrhage; disability after one year was reported in 11
infants (9%). Of those cases with an adverse outcome, only 23% had a known family
history of FMAIT. The incidence of clinically detected FMAIT estimated from this national
study is less than one sixth of that estimated from prospective screening studies. Almost
three quarters of cases with serious clinical problems did not have a known family history
of FMAIT, highlighting the importance of appropriate assessment of the case for antenatal
screening. This study represents a major contribution to the epidemiology of FMAIT in the
UK.
Source: EMBASE
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56. Successful management of term pregnancy complicated with deep vein
thrombosis and heparin-induced thrombocytopenia
Author(s): Sioulas V., Mourtzakis S., Salamalekis G., Karanikolopoulos P., Chrelias C.,
Grouzi E., Brountzos E., Kassanos D.
Citation: Journal of Maternal-Fetal and Neonatal Medicine, May 2010, vol./is. 23/(211),
1476-7058 (May 2010)
Publication Date: May 2010
Abstract: Brief Introduction: The aim of this study is to present a case of deep vein
87
thrombosis (DVT) and heparin-induced thrombocytopenia type II (HIT II) during term
pregnancy, treated with inferior vena cava (IVC) filter insertion and fondaparinux
administration. Clinical Cases or Summary Results: A 31-yearold nulliparous woman at 37
weeks of gestation was referred to our Department for further management of left
popliteal vein thrombosis. Her medical history was significant for systemic lupus
erythematosus and a prior episode of DVT. During the course of pregnancy, she was
treated with azathioprine, dexamethasone and tinzaparin (4500 U/24 h). On admission,
the woman was switched to IV infusion of unfractionated heparin, but, 24 h later, a
marked decrease in platelet count was recorded. Functional and ELISA assays confirmed
the diagnosis of HIT. An IVC filter was inserted and the patient underwent cesarean
section. A baby boy weighing 2510 gr, with umbilical artery pH of 7.32 and Apgar score of
7/1'9/5', was delivered. Postpartum anticoagulation consisted of fondaparinux (7.5 mg/24
h), gradually replaced by oral anticoagulants, for a period of 6 months. IVC filter was
removed 5 weeks after deployment. Conclusions: Fondaparinux, an alternative option for
the treatment of HIT, may be safely used in pregnant or lactating women. However, when
therapeutic anticoagulation is contraindicated or fails, the placement of IVC filter during
pregnancy complicated with DVT is not, probably, associated with adverse maternal or
fetal outcomes.
Source: EMBASE
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57. Thrombocytopenia during pregnancy - More than one disease
Author(s): Vladareanu R., Nicolescu A., Vladareanu A.M.
Citation: Journal of Maternal-Fetal and Neonatal Medicine, May 2010, vol./is. 23/(76),
1476-7058 (May 2010)
Publication Date: May 2010
Abstract: Thrombocytopenia, complicating up to 10% of all pregnancies may result from
very many causes. The presence of thrombocytopenia in pregnant women demandes to
exclude false thrombocytopenia and imposes the need for a correct clinical, etiological
and serological diagnosis in order to apply a better treatment and also provide a better
monitoring of the pregnancy. Distinction between ITP and gestational thrombocytopenia
may be difficult when thrombocytopenia is first discovered during pregnancy. Autoantibodies are the hallmark of autoimmunity, and they are not found in gestational
thrombocytopenia. In case of ITP, the detection of the antiplatelet antibodies may be
useful in monitoring the evolution of the disease and treatment efficiency. Serious
maternal risks are uncommon, therefore more conservative management should be
applied. However, the therapy is required if thrombocytopenia is below 50x109/L or
hemorrhagic syndromes occurs. Maternal therapy implies: Corticosteroids (later response;
safe for the fetus and adverse events for the mother); Intravenous immunoglobulins
(rapid response, indicated in the predelivery period or in case of failure to corticosteroids),
splenectomy (rarely done in 2nd trimester) and new therapies (Romiplostim, Rituximab).
Short-term administration of the thrombopoesis-stimulating protein, Romiplostim, has
been shown to increase platelet counts in most patients with ITP. Transplacental passage
of maternal autoantibodies could lead to fetal/ neonatal thrombocytopenia. The
characteristics of the biological maternal parameters may be predictive for the
assessment of possible fetal or neonatal complications (intracerebral haemorrhage appear
only in 0-3% of cases). Close monitoring is necessary.
Source: EMBASE
Full Text:
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58. Prenatal diagnosis of fetal intracranial hemorrhage in pregnancy complicated by
idiopathic thrombocytopenic purpura
Author(s): Koyama S., Tomimatsu T., Sawada K., Kanagawa T., Isobe A., Taniguchi Y., Wada
T., Kimura T., Arahori H., Kitabatake Y., Wada K.
Citation: Prenatal Diagnosis, May 2010, vol./is. 30/5(489-491), 0197-3851;1097-0223 (May
2010)
Publication Date: May 2010
Source: EMBASE
59. Retrospective comparison of maternal vs. HPA-matched donor platelets for
treatment of fetal alloimmune thrombocytopenia
Author(s): Giers G., Wenzel F., Fischer J., Stockschlader M., Riethmacher R., Lorenz H.,
Tutschek B.
Citation: Vox Sanguinis, February 2010, vol./is. 98/3 PART. 2(423-430), 0042-9007;14230410 (February 2010)
Publication Date: February 2010
Abstract: Background and Objectives: In fetal alloimmune thrombocytopenia (FAIT),
transplacental maternal antibodies cause destruction of fetal platelets. FAIT is similar to
fetal Rhesus haemolytic disease, but half of the affected fetuses are born to primiparous
women. In 10-20% of cases, prenatal and perinatal intracranial haemorrhages are
reported. Different therapeutic approaches have been described, including maternally
administered high-dose intravenous immunoglobulin (high dose IVIG) without or with
steroids or intrauterine transfusion (IUT) of compatible platelets. For the latter, the use of
plasma-free maternal and donor platelets has been described, but a comparison of these
two sources of platelets has not been reported. Materials and Methods: We
retrospectively analyzed the clinical courses of cases with FAIT treated with IUT of either
HPA-matched donor platelets or maternal platelets, done by a single team between 1990
and 1997. In 57 pregnancies, FAIT was treated by repeated IUT with either maternal (15
fetuses) or donor platelets (42 fetuses). Results: There was no procedure-related fetal or
neonatal loss. Platelets from both sources reliably raised the fetal platelet counts. Donor
platelet preparations contained more platelets and yielded higher fetal post-transfusion
platelet counts, but maternal platelets were clinically equally effective. Conclusions:
Donor and maternal platelet concentrates are effective sources for the treatment of FAIT.
2009 The Author(s).
Source: EMBASE
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60. Antithrombin concentrate as a treatment of preeclampsia induced thrombopenia:
Thromboelastometric and biological data
Author(s): Ducloy-Bouthors A.S., Tournoys A., Wibaut B., Deruelle P., Bauters A.
Citation: Pathophysiology of Haemostasis and Thrombosis, 2010, vol./is. 37/(A82), 14248832 (2010)
Publication Date: 2010
Abstract: Preeclampsia (PE) is a major cause of maternal death. Thrombopenia is observed
89
in 15% of the severe PE and may be explained by immunologic or microthrombotic or
microangiopathic comsumptive mechanisms. Drastic decrease in platelet count is a
criteria for pregnancy medical termination. In this case report, a severe PE with
thrombopenia and acquired antithrombin (AT) deficiency was treated with AT concentrate
(Aclotine LFB France) and monitored by thromboelastometry (ROTEM Pentapharm
Munich). Case: Mrs V.S . 5pare, 38SG, 28 years old, BMI 39 kg/m2, was admitted in a
tertiary care obstetric unit for severe preeclampsia: moderate hypertension, epigastric
pain, platelet count: 37,109/mm3, elevated hepatic enzymes >10 N, hemolysis. Induction
of labor was decided. Laboratory results and ROTEM showed a hypercoagulable state and
AT deficiency 40%. (CT and CFT were decreased, alpha angle and MCF in normal range and
FIBTEM amplitude increased). AT concentrate (Aclotine LFB France) [1000 UI/30 minutes
then 2000 UI/12h] was administered in order to obtain AT activity 72% and platelet count
99 109/mm3. Haemostasis remained in the normal range for the course of labor and
caesarean section (Table). No postpartum haemorrhage HPP=950 ml was observed. Postpartum treatment was nicardipine and enoxaparine 26000 UI/j. Mother and child
discharged at Day 5 from from the hospital without complication. Decrease in AT activity
is known to predict the severity of preeclampsia. Terao and Paternoster have studied the
benefit of AT administration on the course of maternal and foetal disease (2, 3). However
AT is used carefully because of a potential increase in hemorrhagic risk. In this case report,
hypercoagulability, AT efficacy and safety are documented by thromboelastometry. Table
presented here.
Source: EMBASE
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61. Thrombotic thrombocytopenic purpura (TTP) and pregnancy: Presentation and
management in subsequent pregnancies
Author(s): Thomas M.R., Camilleri R.S., MacHin S.J., Scully M.A.
Citation: Pathophysiology of Haemostasis and Thrombosis, 2010, vol./is. 37/(A4), 14248832 (2010)
Publication Date: 2010
Abstract: Background/Aims: Half of all patients with acute TTP are women of child bearing
age. Increasing use of ADAMTS13 assays and mutational analysis has improved pregnancy
management. Materials and Methods: All UK cases of TTP referred for ADAMTS13 analysis
or managed at UCLH were included. Results: 8/16 cases of acute TTP presenting in
pregnancy had congenital TTP. Diagnosis was confirmed by ADAMTS13 activity <5%, no
anti-ADAMTS13 IgG and homozygous/ compound heterozygous/heterozygous gene
defects. The most commonly detected abnormality was located in exon 24, affecting the
distal end of the ADAMTS13 protein. Presentation was in the 1st trimester (n=1), 2nd
trimester (n=4) and 3rd trimester (n=3). Fetal deaths in untreated or presenting
pregnancies were 6/11 (twins x1). Subsequent pregnancies were treated throughout with
intermediate-purity FVIII, plasma infusion or plasma exchange (PEX) with live births in 5/7,
although pregnancy-related complications such as hypertension were increased. 8 further
women had pregnancy-associated TTP, most with anti- ADAMTS13 IgG. The majority
presented in the 3rd trimester/ postpartum (n=5). We have managed 13 pregnancies in
women with a past history of TTP including 2 sets of twins. All received low dose aspirin
(LDA)+/-LMWH prophylaxis. ADAMTS13 activity was monitored regularly. Women with
normal first trimester ADAMTS13 did not have TTP-associated complications. Elective PEX
was required in one pregnancy when levels fell to 10-15%. Regular fetal US+/-uterine
artery dopplers suggested management was satisfactory. There were no fetal deaths.
Conclusions: Late onset congenital TTP must be considered when the disease presents in
90
pregnancy. Women with congenital TTP require therapy throughout pregnancy. In women
with previous acquired TTP, baseline ADAMTS13 activity and antibody status may identify
likely relapse. Elective PEX should be considered in women with reduced ADAMTS13 (<1015%) and/or raised IgG. LDA+/-prophylactic LMWH are used to reduce complications
related to placental thrombosis. Successful pregnancy outcomes are possible in both
acquired and congenital TTP.
Source: EMBASE
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62. International consensus report on the investigation and management of primary
immune thrombocytopenia
Author(s): Provan D., Stasi R., Newland A.C., Blanchette V.S., Bolton-Maggs P., Bussel J.B.,
Chong B.H., Cines D.B., Gernsheimer T.B., Godeau B., Grainger J., Greer I., Hunt B.J.,
Imbach P.A., Lyons G., McMillan R., Rodeghiero F., Sanz M.A., Tarantino M., Watson S.,
Young J., Kuter D.J.
Citation: Blood, January 2010, vol./is. 115/2(168-186), 0006-4971;1528-0020 (14 Jan
2010)
Publication Date: January 2010
Abstract: Previously published guidelines for the diagnosis and management of primary
immune thrombocytopenia (ITP) require updating largely due to the introduction of new
classes of therapeutic agents, and a greater understanding of the disease
pathophysiology. However, treatment-related decisions still remain principally dependent
on clinical expertise or patient preference rather than high-quality clinical trial evidence.
This consensus document aims to report on new data and provide consensus-based
recommendations relating to diagnosis and treatment of ITP in adults, in children, and
during pregnancy. The inclusion of summary tables within this document, supported by
information tables in the online appendices, is intended to aid in clinical decision making.
2010 by The American Society of Hematology.
Source: EMBASE
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63. Thrombotic thrombocytopenic purpura: Recognition and management
Author(s): Kiss J.E.
Citation: International Journal of Hematology, January 2010, vol./is. 91/1(36-45), 09255710 (January 2010)
Publication Date: January 2010
Abstract: Thrombotic thrombocytopenic purpura is a life-threatening multisystem
disorder that represents both a diagnostic and a management challenge to clinicians. Early
recognition of the condition coupled with rapid institution of plasma exchange has led to a
dramatic improvement in prognosis. Studies performed over the past decade have
elucidated the predominant pathophysiology, stemming from a deficiency of ADAMTS13,
that accounts for the widespread microvascular deposition of platelet-von Willebrand
factor in many sites, including the brain, kidney, and mesenteric vessels. However, in light
of the mortality rate of 10-20%, much work remains to be done to translate advances in
our understanding of pathophysiology into clinical practice. Improvements in medical
management using immunosuppressive and other drugs are being actively explored in
91
clinical trials. Agents that target ADAMTS13 autoantibody production by B-cells, such as
anti-CD20 monoclonal antibodies, have the potential to shorten the duration of plasma
exchange treatment, reduce relapses, and transform the management of this once
enigmatic disorder. 2010 The Japanese Society of Hematology.
Source: EMBASE
64. Pathophysiology of thrombotic thrombocytopenic purpura
Author(s): Tsai H.-M.
Citation: International Journal of Hematology, January 2010, vol./is. 91/1(1-19), 09255710 (January 2010)
Publication Date: January 2010
Abstract: Thrombotic thrombocytopenic purpura (TTP) is a disorder with characteristic
von Willebrand factor (VWF)-rich microthrombi affecting the arterioles and capillaries of
multiple organs. The disorder frequently leads to early death unless the patients are
treated with plasma exchange or infusion. Studies in the last decade have provided ample
evidence to support that TTP is caused by deficiency of a plasma metalloprotease,
ADAMTS13. When exposed to high shear stress in the microcirculation, VWF and platelets
are prone to form aggregates. This propensity of VWF and platelet to form microvascular
thrombosis is mitigated by ADAMTS13, which cleaves VWF before it is activated by shear
stress to cause platelet aggregation in the circulation. Deficiency of ADAMTS13, due to
autoimmune inhibitors in patients with acquired TTP and mutations of the ADAMTS13
gene in hereditary cases, leads to VWF-platelet aggregation and microvascular thrombosis
of TTP. In this review, we discuss the current knowledge on the pathogenesis, diagnosis
and management of TTP, address the ongoing controversies, and indicate the directions of
future investigations. 2010 The Japanese Society of Hematology.
Source: EMBASE
65. Thrombocytopenia in pregnancy
Author(s): McCrae K.R.
Citation: Hematology / the Education Program of the American Society of Hematology.
American Society of Hematology. Education Program, 2010, vol./is. 2010/(397-402), 15204383 (2010)
Publication Date: 2010
Abstract: Thrombocytopenia occurs commonly during pregnancy, and may result from
diverse etiologies. Awareness of these many causes facilitates proper diagnosis and
management of thrombocytopenia in the pregnant setting. Some causes of
thrombocytopenia are unique to pregnancy and may not be familiar to hematologists. In
the review, we will discuss the differential diagnosis of thrombocytopenia in pregnancy,
and the pathogenesis of selected thrombocytopenic disorders. Considerations for optimal
management of the pregnant patient with thrombocytopenia will also be described.
Source: EMBASE
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66. High LDH to AST ratio: Rapidly available aid to support a suspected diagnosis of
pregnancy-related thrombotic thrombocytopenic purpura
Author(s): Keiser S., Boyd K., Rehberg J., Elkins S., Owens M., Martin J.
92
Citation: American Journal of Obstetrics and Gynecology, December 2009, vol./is. 201/6
SUPPL. 1(S286), 0002-9378 (December 2009)
Publication Date: December 2009
Abstract: OBJECTIVE: Thrombotic thrombocytopenic purpura (TTP) is rarely encountered
during pregnancy or the puerperium. Diagnosis is challenging as TTP can mimic severe
preeclampsia/HELLP syndrome; definitive laboratory testing remains protracted and
problematic. This study sought to explore the disease course, treatment, maternalperinatal outcomes and the LDH-to-AST ratio findings in a single institution series of
gestational/puerperal patients considered to have TTP. STUDY DESIGN: Retrospective
review of all pregnant/puerperal patients with TTP from a single tertiary care center
during 1986-2006. Data were collected and analyzed, and are reported as median (range)
or percentage. RESULTS: 13 pregnant (N=10) or puerperal (N = 3) patients with TTP were
identified; 11 cases were primary, 2 were recurrent. Gestational age at diagnosis for
pregnancy cases was 36 (22-39) weeks. Most TTP patients in this series (53.9%) were
considered initially to have severe preeclampsia/HELLP. Three (23.1%) were febrile and
one (7.7%) had seizures. Laboratory findings included an LDH to AST ratio of 61 (22-185),
indirect bilirubin 1.65 (0.66-4.68) mg/dL, peak serum creatinine 1.60 (0.7-9.6) mg/dL,
platelet nadir 18 (7-105)K/uL, and schistocytes with hematuria were uniformly present.
ADAMTS-13 values were not available. No patient improved in response to delivery. Two
maternal (15.4%) and eight perinatal (61.5%) deaths occurred. Four patients subsequently
developed chronic renal failure. All TTP patients underwent serial plasma exchanges (PEX),
92.3% received corticosteroids, 69.2% required antihypertensives and 23.1% received
sulfinpyrazone. CONCLUSION: Because pregnancy-associated TTP is associated with a
significant risk of morbidity and mortality for both patient and progeny, emergently
differentiating between preeclampsia/HELLP syndrome and TTP is critical. Until rapid,
sensitive and specific testing for TTP is readily available, we recommend considering a
very high LDH to AST ratio, a very low platelet count and hematuria to be suggestive of
TTP for emergency PEX management purposes.
Source: EMBASE
67 Advances in alloimmune thrombocytopenia laboratory investigations
Author(s): Kaplan C.
Citation: Vox Sanguinis, November 2009, vol./is. 97/(24), 0042-9007 (November 2009)
Publication Date: November 2009
Abstract: Alloimmune thrombocytopenia is mainly encountered during pregnancy when
the mother becomes immunized against the fetal platelets antigens she lacks. Therefore
during pregnancy the maternal IgG cross the placenta and recognize their target on the
fetal platelets. The resulting fetal alloimmune thrombocytopenia, which incidence has
been evaluated to 1/ 1000 live births in Caucasians, is usually severe and can lead to
bleeding. Intracranial hemorrhage (20-30% in retrospective studies) is the most severe
complication leading to death (10-15%) or neurological sequelae (20%). Due to the
recurrence for incompatible fetuses in the subsequent pregnancies, antenatal
management has been developed. The diagnosis of fetal or neonatal alloimmune
thrombocytopenia is of utmost importance for the management of the affected infant and
the subsequent pregnancies. The diagnosis is straightforward when a maternal
alloantibody is detected directed against the offending antigen present in the infant. The
molecular basis of the platelet alloantigens has been elucidated and single nucleotide
polymorphism (SNP) in the gene encoding the relevant glycoprotein is present in the
23/24 defined antigens. Antigen determination has evolved during the recent years. A
number of genotyping methods have been developed: PCR-RFLP, PCR-SSP, real time PCR
and more recently microarrays. Although genotyping is widely used, unknown genetic
93
variants may alter the results, and ethnic diversity is of importance. Genotype is not
always phenotype. However phenotyping is somehow problematic because reference
human sera have limited availability. The detection of the alloantibodies could be
challenging. Currently the most widely techniques used are the antigen-capture assays
with mouse monoclonal antibodies. However false-negative results may occur by steric
hindrance between the human antibody and the monoclonal antibody. Excessive washing
procedure may result in the dissociation of low avidity alloantibodies. In addition
modification of the epitope during storage should be taken into account. Alloantibodies
are heterogeneous, anti HPA-1a undetectable with the MAIPA technique HPA-1a, << false
negative MAIPA >>, are detectable with surface plasmon resonance technology. Some anti
HPA-3a alloantibodies are detectable only using fresh platelets in flow cytometry assays.
Detection of alloantibodies directed against low frequency antigens is somehow more
complicated. Cross-match with maternal sera and paternal platelets is to be performed.
However it depends on paternal platelets availability and ABO mismatch. New techniques
are developed to overcome these problems such as B-lymphoblastoid cell lines, stably
transfected CHO cells and recombinant mini-b3 integrin fragments. Due to variation in
sensitivity of antibody detection among laboratories and the absence of monoclonal
antibodies against human platelet antigens, only HPA-1a antibodies have been made up to
now, international standard reagents for detection of human antibody against human
platelet antigen have also been developed: Anti HPA-1a for quantification, anti HPA-5b,
anti HPA-3a and minimum sensitivity for anti HPA-1a. Difficulties in laboratory diagnosis
for fetal and neonatal alloimmune thrombocytopenia should not delay therapy when
there is bleeding tendency or severe thrombocytopenia. When the diagnosis is equivocal,
retesting is recommended with new samples. In conclusion, international workshop
exercises are of importance for further improvement and quality assurance proficiency.
Source: EMBASE
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68. Therapy for chronic ITP in Germany - A patient survey
Author(s): Matzdorff A.C., Arnold G., Salama A., Ostermann H., Hummler S.
Citation: Blood, November 2009, vol./is. 114/22, 0006-4971 (20 Nov 2009)
Publication Date: November 2009
Abstract: Background: Guidelines recommend glucocorticoids and splenectomy as
standard 1st and 2nd line treatments for chronic ITP. We sought to find out how German
ITP-patients are treated in respect of these guidelines. Methods: Members of a patient
support association >18 y with self-reported history of chronic ITP (>6 mo) were surveyed.
A questionnaire was developed from literature review with clinician and patient input,
and administered on-line. Results: 123 questionnaires were evaluated. Age (median 51
years) and gender distribution (38% m, 62% f) are comparable to surveys from other
countries. 70% of patients had chronic ITP for more than 5 years and 50% a usual platelet
count of < 50.000/6l (20% < 30.000/mul). 69% had hematomas or petechiae within the
last 12 months, 45% had oropharyngeal bleeds, and 11% had been admitted to a hospital
within this year. 88% had received or receive glucocorticoids, 28% were splenectomized.
IVIg was given to 55%, rituximab to 22%, anti-D to 11%, cyclosporine to 7%.
Complementary and alternative medical treatments had been used by 36%. 38 women
were under the age of 50 and 14 (36%) reported that they had been advised not to
become pregnant. 23 became pregnant and 10 (44%) required ITP-treatment during their
pregnancy. Conclusion: Glucocorticoids are the most common therapy for chronic ITP but
complementary and alternative treatments already come second and less than 1/3 of the
patients are splenectomized. This and the frequent use of complementary medicines
94
suggests dissatisfaction with conventional therapeutic approaches. Many patients receive
off-label therapies (rituximab, anti-D, cyclosporine are not licensed for ITP in Germany).
There is a major need for adequate counseling and care for pregnant ITP-patients.
Source: EMBASE
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69. Impact of pregnancy on the course of immune thrombocytopenic purpura: An
observational study on 44 cases
Author(s): Baili L., Khellaf M., Languille L., Bierling P., Godeau B., Michel M.
Citation: Blood, November 2009, vol./is. 114/22, 0006-4971 (20 Nov 2009)
Publication Date: November 2009
Abstract: Backgound: Adult's immune thrombocytopenic purpura (ITP), now referred as
immune thrombocytopenia, is an autoimmune disease affecting preferentially women of
child-bearing age. The risk of relapse or worsening of the disease during pregnancy in
women with a previous history of ITP or followed for a chronic ITP is not well known and
the monitoring of such patients is therefore not consensual. In order to better asses the
impact of pregnancy on ITP' course and natural history, a study was initiated at the
national referral center for adult's immune cytopenias at Creteil, France. Patients and
Methods: This was an observational single center study. To be included into the study, all
women had to fulfill the following inclusion criteria: 1) A previous history of definite ITP
outside pregnancy with a platelet count < 50x109/L at time of diagnosis and 2) Occurrence
of at least one pregnancy within 10 years after ITP diagnosis. Patients diagnosed with
secondary ITP (lupus-associated or other) or in whom ITP was diagnosed during a previous
pregnancy could not be included. All available clinical and biological ITP-related data
available before, during and after each pregnancy were extensively reviewed and
analyzed. Results: Data on 44 pregnancies in 33 women (mean age: 25 +/- 7 years) were
analyzed. The mean delay between ITP diagnosis and first pregnancy was 52 +/- 19,8
months. At the beginning of pregnancy, ITP was considered active (i.e platelet count
<100x109/L) in 11/44 (25%) cases, with a platelet count below 50 x109/L in 6 cases
whereas ITP was in remission (platelet count > 100 x109/L) in 75% of the cases, either off
therapy (82%) or on treatment (18% of the cases). In total, the platelet count remained
stable during pregnancy In 25/44 of the cases (57%) without the need of any treatment
except for one patient who received corticosteroids for an associated autoimmune
hemolytic anemia diagnosed during pregnancy (Evans' syndrome). A slight decrease in the
platelet count (between 50 and 100x109/L) was observed in 12 cases (27%), 6 of which
occurred at the end of pregnancy. In nine of these cases, patients were given a short
course of corticosteroids in preparation for delivery. Lastly, a decrease of the platelet
count below 50x109/L was observed in only 7 of the 44 pregnancies (16%). In 6 of these 7
cases, patients were given corticosteroids, either alone (n=2) or in combination with
intravenous immunoglobulin (n=4 cases); one patient was also given a platelet
transfusion. No severe bleeding episode (mucosal bleeding or any hemorrhage) occurred
in any of these cases prior to, during or after delivery. A miscarriage occurred in 6 of the
44 pregnancies (13.5%), a C-section was performed in 18% of the cases which is the usual
average rate in France. In total, a treatment for ITP had been considered useful in 15
pregnancies (34%) mainly at the end of the third trimester. The mean platelet count at
time of delivery was 107 +/- 17 x109/L, None of the patients had a relapse or a significant
decrease of the platelet count within 6 months after delivery except for a one patient who
presented with a severe (platelet count < 20 x109/L) and symptomatic (cutaneous and
mucosal bleeding) thrombocytopenia on day 2 after delivery. Conclusion: Based on these
preliminary data, pregnancy does not seem to have a negative impact on the course of the
95
disease in women with chronic non-refractory ITP nor to increase the risk of relapse in
those with a previous history of ITP. A significant decrease of the platelet count may occur
in about 15% of the cases, mainly during the third trimester. In women with a platelet
count between 50 and 100 x 109/L at term, a short course of treatment could be indicated
in preparation for delivery and especially if an epidural analgesia is planned.
Source: EMBASE
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70. Intracranial hemorrhage in alloimmune thrombocytopenia: Stratified
management to prevent recurrence in the subsequent affected fetus
Author(s): Bussel J.B., Wissert M., Tsaur F.W., Hung C., Primiani A.
Citation: Blood, November 2009, vol./is. 114/22, 0006-4971 (20 Nov 2009)
Publication Date: November 2009
Abstract: Introduction: Fetal and neonatal alloimmune thrombocytopenia (AIT) result
from parental platelet-specific antigen incompatibility combined with maternal
sensitization. AIT is the most common cause of severe thrombocytopenia and intracranial
hemorrhage (ICH) in term newborns; if ICH occurs in 1 child then the next affected sibling
has a > 90% chance of developing an ICH. Prevention of recurrent ICH is the primary goal
of antenatal management of AIT. Methods: Thirty-three women with 37 separate
pregnancies were enrolled in 2 consecutive studies of antenatal management of AIT. All
patients had a previous child who had suffered an ICH. They were subdivided into 3
groups (Extremely High Risk (EHR), Very High Risk (VHR) and High Risk (HR)) based on the
timing of that child's ICH. Therapy was stratified according to the timing of the sibling ICH.
The table below lists risk stratification, treatment and outcomes for each treatment arm.
In all arms, if fetal blood sampling (FBS) showed a platelet count < 30,000/mL3 or if FBS
was not performed, therapy was intensified with additional prednisone and/or
intravenous IVIG. Results: The mean, on-therapy fetal platelet count was greater than the
previous sibling birth platelet count (BPC) in all arms. The first EHR fetus, whose mother
received IVIG 1g/kg/wk and prednisone 1mg/kg/d beginning at week 12, suffered an ICH
at 19 weeks gestation and died. The EHR protocol was changed to IVIG 2g/kg/wk. None of
the 7 other EHR fetuses had an ICH, and all had BPC > 50,000/mL3 . Two patients in the
VHR group had ICHs, but both occurred at BPC > 100,000/mL3 . One was a Grade I (of IV)
ICH; the other occurred in a 24-week-old premature infant. Fourteen of the 17 VHR
fetuses had BPC > 50,000/mL3 . Within the HR group, 2 fetuses (therapy started at 20-24
weeks), suffered an ICH. One patient should have been treated in the VHR arm, but could
not receive optimal treatment due to late referral. The other ICH was Grade I, although
the BPC was low. All HR fetuses, except for 3 receiving IVIG 1g/kg/wk starting at 20-24
weeks, had BPC > 50,000/mL3 . Major complications of the study were FBS-related: 1 case
of fetal tachycardia and 8 cases of fetal bradycardia; 3 cases lead to emergent cesarean
section, one a result of hemorrhaging from the needle insertion site. Discussion: Fetuses
of AIT-affected pregnancies with ICH in a previous affected sibling are at very high risk for
ICH. The results of this study demonstrate the success of risk-tailored treatment in
prevention of recurrent ICH. Although all or almost all 37 of the fetuses in this study would
have developed a recurrent ICH, there were only 5 recurrent ICHs and only 2 of 5 occurred
due to failure of therapy. Treatment should be stratified based on the timing of the sibling
ICH: the highest risk is second trimester antenatal hemorrhage followed by third trimester
followed by perinatal. See the table below for recommended treatments for each group.
Since complications due to FBS continued to be substantial, FBS should be avoided until
after 32 weeks at which time urgent delivery could be performed if a complication
occurred. The intensification of treatment at fixed gestational ages rather than relying on
96
the results of FBS further minimizes the need for early FBS. Risk Stratification, Treatment,
Outcome and Recommendations (Table prsented) ICH, intracranial hemorrhagt; IVIG,
intravenous immunoglobulin; Pred, prednisone; BPC, birth platelet count; FPC, fetal
platelet count; FBS, fetal blood sampling.
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71. Therapeutic plasmapheresis and intravenous immunoglobulin as a treatment
modality in a 45 year old pregnant female with refractory idiopathic thrombocytopenic
purpura: A case report
Author(s): Giovanniello D.S., Chiofolo J.T., Grima K.
Citation: Transfusion, September 2009, vol./is. 49/(77A), 0041-1132 (September 2009)
Publication Date: September 2009
Abstract: Background: Idiopathic thrombocytopenic purpura (ITP) is a clinical syndrome in
which a decreased number of circulating platelets manifests as a bleeding tendency, easy
bruising, or extravasation of blood from capillaries into skin and mucous membranes. In
patients with ITP, platelets are coated with autoantibodies (usually IgG) to platelet
membrane antigens, resulting in splenic sequestration and destruction. The resulting
shortened life span of platelets in circulation results in a decreased platelet count. The
platelet antibodies and ensuing reduction in platelet count in pregnancy can increase
morbidity and mortality secondary to maternal hemorrhage at time of birth and can result
in neonatal thrombocytopenia, secondary to maternal antibodies crossing the placenta.
Neonatal thrombocytopenia places the infant at risk for intracranial or visceral
hemorrhage. Treatment options in ITP patients usually include corticosteroids and
intravenous immunoglobulin (IVIG), with splenectomy in more chronic disease conditions.
Therapeutic plasmapheresis has not been viewed as beneficial for use in ITP patients,
secondary to the rapidly equilibrating interstitial antibodies present in this disease. The
addition of IVIG may prevent this equilibrating rebound phenomenon. There are several
case reports which support this combination and one small trial showing that the
combination of plasmapheresis and steroids was beneficial in decreasing relapse rate
(Transfusion 1981;21:291- 8), suggesting that the addition of plasmapheresis may provide
some benefit. Case Report: We present a case report of a 45 year old, 10 week pregnant,
female with a history of two failed pregnancies, at least one of which failed at 14 weeks
gestation, and ITP refractory to treatment with high doses of prednisone and IVIG. The
admission platelet count was 16,000/?L. Within 24 hours, the platelet count dropped to
2,000/?L. The patient was treated with a combination of three plasmapheresis procedures
where 3 liters of plasma were removed and replaced with 2 liters 5% albumin and 1 liter
of FFP, followed by 80 mg/kg IVIG weekly. The patient's platelet count subsequently rose
to 41,000/?L upon discharge. The platelet count remained stable for the remainder of the
pregnancy (40-50,000/?L) while being maintained on prednisone 80 mg and IVIG 80 mg/kg
weekly at home. She delivered a normal baby with a normal platelet count of 272,000/?L
at 31 weeks gestation by Cesarean section, prematurely secondary to preterm premature
rupture of membranes (PPROM). Conclusion: Patients with refractory ITP may benefit
from a combination of therapeutic plasmapheresis and IVIG. In pregnancy, this reduces
the risk of hemorrhage to both the mother and infant.
Source: EMBASE
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72. Successful treatment of a pregnant woman with active systemic lupus
erythematosus (SLE) and secondary idiopathic thrombocytopenic purpura (ITP) after 3
sessions of plasma exchange: A case report
Author(s): Hussein E.A., Sameeh I.A.
Citation: Transfusion, September 2009, vol./is. 49/(76A-77A), 0041-1132 (September
2009)
Publication Date: September 2009
Abstract: Background: There is controversy about the therapeutic efficacy of plasma
exchange (PE) in patients with active SLE. Case report: We report a case of a 26-year old
female, pregnant at 24 weeks, presenting with pallor, vaginal bleeding, bleeding gums,
facial rash, blurry vision, and lymphadenopathy. She was admitted to our hospital with
pancytopenia and received packed red cells and 18 units of platelets. A diagnosis of SLE
with secondary ITP was made based on her laboratory findings which revealed;
leucopenia, thrombocytopenia with a platelet count of 6,000/?L, evidence of hemolysis
with hyperbilirubinemia and a hemoglobin level of 5 gm/dl, positive antinuclear antibody,
positive anti double- stranded- DNA antibody, positive lupus anticoagulant, false positive
VDRL, and decreased complement levels. Direct coombs' test was negative and her LDH
(lactate dehydrogenase) was 529 IU/L. The patient had normal coagulation, and renal
parameters. TTP (thrombotic thrombocytopenic purpura) was ruled out when no
schistocytes were identified manually in the peripheral blood smear. Pre-eclampsia,
eclampsia, and HELLP (hemolysis, elevated liver enzymes and low platelet count)
syndrome were also excluded. High doses of corticosteroids were given when her illness
was worsening with persistent hemolysis and thrombocytopenia. Therapy included 3 daily
intravenous pulse doses of methyl prednisolone 500 mg, followed by a daily maintenance
dose of methyl prednisolone 40 mg. She also received cyclosporine 75 mg given twice
daily. Plasma exchange was conducted when she was not responding to the conventional
therapy. Rapid and excellent responses were achieved when 3 PE sessions were carried
out in combination with the previous therapy. Sessions were performed on alternate days
with COBE Gambro machine using albumin and exchanging one plasma volume per
session. No active hemolysis was noted and her facial rash resolved. The patient improved
clinically, her platelet count rose to 130,000/?L and her antibody titer improved. A
maintenance dose of steroids was prescribed after the sessions, and the dose was
gradually reduced. She is on low dose of oral steroids (10 mg) and remains in good
condition with no signs of hemolysis and with a healthy living fetus. Conclusion: Based on
our case report, Plasma exchange initiated early in combination with steroids might be
beneficial in some patients with therapy resistant active SLE.
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73. Pregnancy-related complications in women with rare production-defect
thrombocytopenias
Author(s): Myers B., Elliott D., Pavord S.
98
Citation: Journal of Thrombosis and Haemostasis, July 2009, vol./is. 7/S2(886), 1538-7933
(July 2009)
Publication Date: July 2009
Abstract: Thrombocytopenia in pregnancy is usually mild and related to increased platelet
consumption rather than production defects. The latter are rare and include hereditary
and acquired thrombocytope-nias, often mistaken for ITP. There is little information on
pregnancy, delivery management and neonatal outcome in these conditions. A
retrospective survey was performed evaluating pregnancy and delivery management in
women with rare platelet production defects. 13 mothers had 32 pregnancies, (18 live
births). The cases included women with May-Hegglin anomaly (3), other hereditary
macrothrombocytopenias (3), TAR syndrome (4) and one each of Fanconi's anaemia,
hypoplastic anaemia & sideroblastic anaemia. Data were collected on platelet count in
pregnancy, antenatal complications, management of delivery, neonatal platelet count,
and post-partum bleeding. Platelet count at booking was 16-120 (mean 56), and mean
platelet count at delivery was 32 x 109/L (range 13-70). There were 13 early miscarriages
(44%) at 6-12 weeks, one termination at 20 weeks (fetus with TAR syndrome) and one
case each of: recurrent antenatal bleeding, pre-eclampsia and IUGR requiring delivery at
31 weeks. Four babies were thrombocytopenic, range (27-49). There were no neonatal
bleeds. Four mothers had been initially diagnosed with ITP and treated with
prednisolone/IVIG with no platelet response in 3. There were 12 vaginal deliveries.
Platelets were given prophylactically in 7 cases, pre-caesarean section or forceps delivery,
and none required as an emergency. Three women had post-partum haemorrhage
(16.6%). In conclusion, although a small group, bleeding and miscarriage rate appeared
increased in this cohort compared to local miscarriage (15%) and PPH (5%) rates. Large
multi-centre studies are required to assess risks and optimal management in this rare and
heterogeneous group.
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74. Maternal deaths due to acquired thrombotic thrombocytopenic purpura (ttp) in
london, england 2003-7
Author(s): Thomas-Dewing R.R., Lucas S.B., Hunt B.J.
Citation: Journal of Thrombosis and Haemostasis, July 2009, vol./is. 7/S2(874), 1538-7933
(July 2009)
Publication Date: July 2009
Abstract: Introduction: TTP is a life threatening disorder more common in women and
occurs in pregnancy. Method: We retrospectively reviewed all maternal deaths due to TTP
in London, confirmed on post mortem examination from 2003 to 2007. The aim of this
review was to identify the mode of death, why they died and identify any preventative
measures that could be taken to prevent future deaths. Results: There were three
maternal deaths in women aged 29, 31 and 35 years and parity 1, 2 and 3 respectively
with no previous complicated pregnancies or TTP, but one had systemic lupus
erythematosus (SLE). Two presented post partum and the third at 24 weeks of gestation.
The first patient was diagnosed with TTP at presentation, but died before treatment was
initiated; the second had a late diagnosis initially thought to have ITP and then HELLP
(haemolysis, elevated liver enzymes, low platelets) syndrome and died while awaiting
transfer to another hospital for plasmapheresis; the third patient was diagnosed with SLE
related complications and a diagnosis of TTP was made post mortem. All three patients
died of sudden cardiac arrest within 1, 5 and 2 days of presentation respectively. Post
99
mortem findings revealed all three patients died with intramyocardial microvascu- lar
thrombosis, the thrombi being characteristically platelet rich (CD61 +, fibrin-). Von
Willebrand factor cleaving protease level (vwf-cp) was not measured in the first, was 0% in
the second and 5% in the third (normal range 66% to 126%) at presentation. Discussion:
This study shows that platelet thrombi in the coronary microvasculature are a significant
cause of early sudden death in TTP in pregnancy, and a reminder of the need to institute
plasmapheresis as soon as possible. If the diagnosis is in doubt especially in differentiating
between the thrombotic microangiopathies of pregnancy, vwf- cp levels should be
collected for measurement, but results not awaited before instituting plasmapheresis.
Source: EMBASE
Full Text:
Available in fulltext at EBSCO Host
75. Gestational thrombocytopenia, is it not simply physiological hypersplenism?
Author(s): Bulvik S., Niven M.J.
Citation: Haematologica, June 2009, vol./is. 94/(526), 0390-6078 (June 2009)
Publication Date: June 2009
Abstract: Background. Thrombocytopenia is an ominous sign during pregnancy. Although
one of the main reason for it is gestational thrombocytopenia, a benign condition
occurring in 8% of all pregnancies and accounting for more than 70% of cases of
thrombocytopenia in pregnancy, there is no pathonomonic sign for it and the diagnosis is
by exclusion of other, sometimes emergent, causes of thrombocytopenia, such as HELLP
syndrome, DIC, Pre Eclampsia, TTP, ITP etc. Aims. To establish a simple test for the
diagnosis of gestational thrombocytopenia. Design and Methods. Nineteen pregnant
women with an eventual diagnosis of gestational thrombocytopenia were investigated
including standard physical examination, blood film, coagulation testing, liver function
tests and abdominal ultrasonography. Results. Of the 19 women, 16 had splenomegaly on
both physical examination and ultrasound. In the remaining 3 spleen size was at the upper
limit of normal on ultrasonography. The reason for this was physiological gestational
splenomegaly, which resolved immediately after the pregnancy. Conclusion. We suggest
that in pregnant women with asymptomatic thrombocytopenia a finding of physiological
splenomegaly strongly suggests the diagnosis of gestational thrombocytopenia. Larger
studies are needed to confirm this hypothesis that gestational thrombocytopenia is simply
due to hypersplenism secondary to the pregnancy.
Source: EMBASE
Full Text:
Available in fulltext at National Library of Medicine
Available in fulltext at Highwire Press
76. Treatment of ttp in pregnancy with therapeutic plasma exchange
Author(s): Nelson G., Raife T., Erikson Y.
Citation: Journal of Clinical Apheresis, 2009, vol./is. 24/2(89), 0733-2459 (2009)
Publication Date: 2009
Abstract: History: The patient is a G2P1 35 year old pregnant female with a history of
thrombocytopenic purpura (TTP). During her first pregnancy in 2003 she presented with
thrombocytopenia, discovered on a routine CBC. She was initially treated with prednisone
for presumed ITP, and underwent emergent cesarean section. Her thrombocytopenia
100
progressed and she was eventually diagnosed with TTP. She was successfully treated with
a prolonged course of therapeutic plasma exchange (TPE). In 2005 she had a recurrent
episode of TTP in which she presented with fever and bruising. There were no known
stimulating factors. She was again successfully treated with TPE. Her ADAMTS13 level was
subsequently found to be undetectable, and she had a high level of ADAMTS13 inhibitor.
During her second pregnancy in 2008, close laboratory monitoring revealed a gradual
decrease in platelet counts. At 26 weeks gestation, she presented with bruising,
thrombocytopenia, and elevated LDH. Severe preeclamptic toxemia with HELLP
(hemolysis, elevated liver enzymes, and lowered platelets) syndrome was ruled out due to
absence of hypertension and AST/ALT and creatinine levels within normal range. Her
pregnancy was otherwise uncomplicated. Methods: TPE was performed using the Cobe
Spectra. The volume removed with each TPE ranged from 3200-4000mL. Replacement
fluid consisted of a combination of 5% Albumin (volume ranged from 250-750mLs),
Normal Saline (volume ranged from 500-1000mLs), and cryo-poor plasma (volume ranged
from 1600-2600mLs). All TPE were maintained at a fluid balance of 95%. During the course
of TPE, the patient received steroid treatments. Fetal monitoring was performed
throughout all exchanges. Results: TPE treatments spanned Jan. 7th -Feb 8th 2008. Daily
TPE was initially performed for eight days. Jan 14th 2008 patient was discharged with plan
of every other day TPE. On Jan 20th the patient was re-admitted due to a decrease in
platelet count. Daily TPE resumed. On Jan 30th TPE tapered to a M-WF schedule. The
infant was delivered Feb 4th 2008 due to fetal distress. The patient received two TPEs
following delivery. The infant was alive and well at completion of TTP treatments.
Discussion: The TPE treatment in conjunction with steroid treatment and close fetal
monitoring maintained the pregnancy through week 30 1/7. Following delivery, the TTP
resolved. This case demonstrates that with appropriate obstetrical care, TPE can
successfully support a TTP patient in the 3rd trimester of pregnancy.
Source: EMBASE
77. Autoimmune thrombocitopenic purpura in pregnancy
Author(s): Christova R., Lisichkov T., Chernev T.
Citation: Akusherstvo i ginekologiia, 2009, vol./is. 48/6(42-46), 0324-0959 (2009)
Publication Date: 2009
Abstract: The author deals with haematologists' and obstetricians' current views on
acquired ATP in children and adults, characterised by a transient, acute or chronic
decrease in platelets count (<50.109/l) due to premature destruction by the
reticuloendothelial system. The most common questions arising in connection with this
disease are: what is autoimmune thrombocytopenic purpura; is there any correlation
between pregnancy and ATP; what are its symptoms; does pregnancy itself affect the
autoimmune disease. If is of utter importance for women with ATP to be aware of the
risks these symptoms pose both on the health of the mother and the foetus. Obstetricians
and gynaecologists seldom object to pregnancy in women with ATP. Nevertheless, it is
essential to point out that additional monitoring and therapy are needed. There is no
medical evidence that supports the notion of terminating pregnancy due to ATP.
Assessment is made only by an obstetrician, haematologist and pediatrician working in
close collaboration. This collaborative work must be present throughout the whole
pregnancy, delivery and puerperium. The treatment necessary for women with ATP aims
to establish platelet count over 50.000 ppm when approaching the end of pregnancy,
preferably between 80.000 ppm - 100.000 ppm taking into account vagina or surgical
delivery as well as the administration of anaesthetic. Delivery management must be
decided entirely on obstetrics consideration, but not on ATP ones. Pregnant women with
ATP must be monitored and treated with caution by a highly specialised medical team.
101
Source: EMBASE
78. Therapeutic approach in pregnant women with an autoimmune
thrombocytopenic purpura
Author(s): Christova R., Lisichkov T., Chernev T.
Citation: Akusherstvo i ginekologiia, 2009, vol./is. 48/6(53-54), 0324-0959 (2009)
Publication Date: 2009
Abstract: The case presented herein aim to update the existing information about the
common diagnostic problems and therapeutic approach in pregnant women that have
autoimmune thrombocytopenic purpura (ATP).
Source: EMBASE
79. Uneventful epidural analgesia in a patient with severe thrombocytopenia
Author(s): Ibrahim S.M., Elgazali M.S.
Citation: Middle East Journal of Anesthesiology, 2009, vol./is. 20/2(291-294), 0544-0440
(2009)
Publication Date: 2009
Abstract: Epidural analgesia is the most effective method for analgesia in labor. It has,
however, contraindications and carries many serious side effects. Though coagulopathy is
an absolute contraindication for epidural and axial blocks, yet there are no absolute limits
for platelet counts that stand in the way of providing epidural analgesia. In a patient who
is writhing in pain due to severe uterine contractions, and in whom there exists a recent
normal platelet screening and no history of bleeding disorders, it is internationally
acceptable between anesthetists to provide epidural analgesia without waiting for a new
platelet screening.
Source: EMBASE
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A new platelet alloantigen, Swia, located on glycoprotein Ia identified in a family with fetal
and neonatal alloimmune thrombocytopenia
H Kroll, K Feldmann, C Zwingel, J Hoch… - …, 2011 - Wiley Online Library
BACKGROUND: Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a bleeding
disorder caused by transplacental passage of maternal antibodies to fetuses whose
platelets (PLTs) express the corresponding human PLT antigen (HPA).
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FNAIT: the fetus pleads guilty!
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thrombocytopenia and of neonatal thrombocytopenia in maternity wards. 1 The ...
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Linking Maternal Platelet Counts with Neonatal Platelet Counts and Outcomes Using the
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102
JD Jensen, SE Wiedmeier, E Henry… - American journal of …, 2011 - thieme-connect.com
... The mothers of three had immune thrombocytopenia purpura (ITP), one had HELLP
syndrome ... 2 Correlation of mother's and neonate's platelet counts when mothers had
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The Parturition Analysis on the Thrombocytopenia of 100 Gravidas
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thrombocytopenia .[J];Current ... Science Beijing 100730, China;Clinical analysis of
idiopathic thrombocytopenic purpura with ...
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Reliable predictors of neonatal immune thrombocytopenia in pregnant women with
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S Koyama, T Tomimatsu, T Kanagawa… - American Journal of …, 2011 - Wiley Online
Library
Of infants born to women with idiopathic thrombocytopenic purpura (ITP), about 10–15%
hve transient neonatal immune thrombocytopenic purpura (NITP). Of concern is the lack
of a reliable predictor for NITP. We conducted a retrospective study of all pregnancies
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Management of thrombocytopenia during pregnancy.
F Boehlen, K Samii… - Revue médicale suisse, 2011 - ncbi.nlm.nih.gov
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Immune thrombocytopenia and pregnancy
S Sankaran… - Obstetric Medicine, 2011 - obmed.rsmjournals.com
Abstract Immune thrombocytopenia (ITP) is not infrequently encountered during
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purpura. ... Number of IUTs in IUT treated neonatesa 3 (1–6) Fetal thrombocytopenia
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OPINION: Some Severe Maternal Diseases Might be Caused by Fetal‐Versus‐Maternal
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L Yan, C Zuo, D Wei… - American Journal of …, 2010 - Wiley Online Library
... liver function tests, low platelet count) syndrome (21%), and immune
103
thrombocytopenic purpura (ITP ... cell transplantation because both of them could cause
maternal thrombocytopenia or ITP. ... 8 found that mild transient thrombocytopenia
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Retrospective comparison of maternal vs. HPA‐matched donor platelets for treatment of
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G Giers, F Wenzel, J Fischer… - Vox …, 2010 - Wiley Online Library
Materials and Methods We retrospectively analyzed the clinical courses of cases with FAIT
treated with IUT of either HPA-matched donor platelets or maternal platelets, done by a
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Foetal and neonatal alloimmune thrombocytopenia (FNAIT)
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Husebekk, A., Skogen, B., Killie, M., Ahlen, T., Tiller, H., Eksteen, M., Stuge, T. and KjeldsenKragh, J.(2011), Foetal and neonatal alloimmune thrombocytopenia (FNAIT). ISBT Science
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