How to take a bleeding history Hemato update 2013 Hospital Ampang Toh See Guan 4 important points 4 important points I wish to obtain from history taking: 1. Establish the presence of bleeding disorder 2. Assess the severity of bleeding 3. Congenital vs acquired 4. Looking for clues associated with specific bleeding disorder Point 1 : Establish the presence of bleeding disorder • Does my patient really has bleeding disorder? • Patients with haemorrhagic disorders always have significant abnormal bleeding histories • Evaluate previous response to hemostatic challenge, e.g. dental extraction, surgery, trauma, childbirth, etc. A significant bleeding history • Epistaxis not stopped by 10 mins compression or requiring medical attention • Cutaneous haemorrhage or bruising without apparent trauma (esp. multiple/ large) • Prolonged (>15 mins) bleeding from trivial wounds, or in oral cavity or recurring spontaneously within 7 days • Post-operative bleeding A significant bleeding history • Menorrhagia (esp. from menarche) – clots > 1 inch in diameter, changing a pad > frequent than 2hourly, or resulting in anemia. • Bruising with minimal or no apparent trauma • Heavy or prolonged bleeding after dental extraction that required medical attention Point 2 : Assess severity of the bleeding • Severe • Minor  Spontaneous haemorrhage  Haemorrhage secondary to  Early onset, usually from major trauma/ surgery infancy  Rare spontaneous  Frequent spontaneous bleed bleed required intervention Point 3 : Congenital vs acquired • Congenital • Acquired  Platelet disorder – Glanzmann thrombasthenia, Bernard Soulier syndrome  Clotting factor deficiency – Haemophilia A & B  Von Willebrand disease  Herediatry haemorrhagic telangiectasia ITP APML/AA/MDS Acquired haemophilia Anticoagulant/ antiplatelet medication  Drug induced thrombocytopenia  Uraemia  Liver disease  DIC     Point 3 : Congenital vs acquired • Congenital • Acquired  Family history – blood relative with bleeding problem; consanguinous marriage; autosomal/Xlinked inheritance  Onset since small/young  Medication history – on anticoagulant/ antiplatelet? medication/ traditional medicine a/w thrombocytopenia  Late/recent onset  Underlying lymphoproliferative d/o, CTD, HIV, HCV, CKD, liver disease, sepsis, etc Inheritance : X-linked recessive Carrier Woman Healthy Man XH X XH X Carrier Girl X X X Healthy Girl XH Y Haemophilic Boy Y X Y Healthy Boy Point 4 : Looking for clues associated with specific bleeding disorder • Mucocutaneous bleed – thrombocytopenias, plt dysfunction, vWd • Cephalhematomas in newborns, hemarthroses, intramuscular, retroperitoneal hemorrhages – severe hemophilias A & B, severe FVII def, severe type 3 vWd, afibrinogenemia Point 4 : Looking for clues associated with specific bleeding disorder • Bleeding from stump of umbilical cord – FXIII def, afibrinogenemia • Recurrent epistaxis & chronic iron def anemia – hereditary hemorrhagic telangiectasia Primary haemostasis EC platelets TF vWF Platelets adhere to vWF-collagen M. Laffan Secondary haemostasis X Xa EC VIIa TF TF-VIIa triggers Xa production Thrombin generation proceeds on PL (platelet) surface M. Laffan Stable clot formation fibrin platelets Stable fibrin-platelet clot is formed M. Laffan Bleeding • Immediate bleeding – Defects in primary haemostasis – Vascular abnormality • Delayed bleeding – Defects in secondary haemostasis A good bleeding history is the best screening test Bleeding disorders not detected by routine coagulation screen • Mild factor deficiencies • von Willebrand disease • Factor XIII deficiency • Platelet disorders • Excessive fibrinolysis • Vessel wall disorders • Metabolic disorders (e.g. uraemia) Case 1 • 1o year old boy with chronic tonsillitis • Planned for tonsillectomy • FBC, PT, APTT sent • Mother c/o that son has easy bruising and recurrent epistaxis and she herself has menorrhagia • Hb 12 Plt 243 • PT 12.5s (12- 16s) APTT 38s (30- 42s) Case 1 – cont’d • Since platelet count and PT, APTT all normal • Mother reassured and proceeded with tonsillectomy • During surgery, excessive bleeding noted but controlled with local measures • 2 hours post-op, further significant bleeding Case 1 – cont’d • Returned to OT, cauterization done • 2 Packed RBC and 2 FFP transfused; bleeding controlled • Repeat PT, APTT the following day- normal • Refer hematologist Case 1 – cont’d • Further bleeding history taken • Mother’s blood sample sent • FBC normal PT 13s (12-16) APTT 40s (3042) • FVIII 34% (40-150) vWF 30% (50-150) • Son’s results similar to mum’s (on f/u) • Diagnosis: von Willebrand disease type 1 Limited investigation of a patient with a bleeding history is as inappropriate as Extensive investigation of a patient with no bleeding history Limitations of PT, APTT • Lack sensitivity and specificity • Tests a very limited portion of haemostasis • Can only detect factor levels below 30% Case 2 • 11 year-old boy • Blunt abdominal trauma by bicycle handle bar on 30th June 2013 • 3 days later on 3rd July – abdominal pain • Hospitalised on 5th July 2013 S. Krishnan 2013 Case 2 – cont’d • In pain • Stable circulation • Tender LHC • Bicycle handle bar impression S. Krishnan 2013 CT scan 05/07/13 S. Krishnan 2013 CT scan 05/07/13 S. Krishnan 2013 CT scan 05/07/13 S. Krishnan 2013 Case 2 – cont’d • Hb 11.6 TW 11.8 • PT 12 s APTT 38.7 s Plt 208 TT 15.7 s • Fibrinogen 2.6 g/L • D-Dimers detected • Serum amylase: not elevated • RP, LFT normal Case 2 – cont’d • 48 hours later … • Increasing abdominal distension and pain • Hb 6.4 g/dL S. Krishnan 2013 CT scan 07/07/13 S. Krishnan 2013 CT scan 07/07/13 S. Krishnan 2013 CT scan 07/07/13 S. Krishnan 2013 CT scan 07/07/13 S. Krishnan 2013 Case 2 – further tests • Repeat tests on 8th July • PT 14 s • APTT 43.4 s • Fibrinogen 4 g/L • Platelet 106 Case 2 – clinical suspicion • Diagnosis: DIC • Rx: Transfusions – Packed red cells – FFP – Platelets and – Cryoprecipitate Case 2 – bleeding history • Normal SVD • No umbilical stump bleeding • Vaccinations – OK • No mucocutaneous bleeds Case 2 – bleeding history • Age 6 years old – delayed expanding haematoma R shin after hit by a stone – Had some tests to investigate bleeding tendency but no abnormalities detected – Surgical evacuation done. No specific therapy – Wound healing by secondary intention S. Krishnan 2013 Arrows mark the linear surgical scar resulting from operative evacuation of an expanding traumatic right shin haematoma at the age of 6 years at Hospital Sultanah Aminah JB Case 2 – family history • 3rd of 5 children; 1 sister and 3 brothers • Non-consanguinous parents • No family history of easy bruising or h/o haemophilia Case 2 – specific factor assays • Factor VIII 13% • Factor IX 150% • vWF Ag 144% • Diagnosis: mild haemophilia A Case 2 – progress • Factor replacement: 100% • Double J stent inserted in L ureter • Planned for evacuation of haematoma Case 3 • 5 year-old boy • Admitted for upper GI haemorrhage • h/o recurrent epistaxis and easy bruising • No f/h of bleeding • Hb 4.5 g/dL TW 4.5 Plt 398 Case 3 – cont’d • PT 12.o (11.5- 14.4) sec • APTT 102.0 (36.9- 45.5) sec • 4 PRBC & 4 FFP transfused • Factor VIII 2.5% • Diagnosis: Moderate Haemophilia A Case 3 – cont’d • Bleeding stopped with FFP x 3 doses • OGDS: pangastritis • Switched to hemofil M (high purity FVIII) • 3 days later, re-bled • Hb fell from 11.o to 5.0 g/dL • APTT 98 sec Mixing studies 48 sec Case 3 - cont’d • Suspected inhibitor; switched to PCC • Unable to do inhibitor assay • Sample sent to reference laboratory – FVIII 3% No inhibitor detected – vWF Ag < 1% • Diagnosis: severe type 3 vWD A good bleeding history is the best screening test Thank you