Liat Mlynarsky, MD
Case presentation
 68 y/o woman
 Presents to the E.R with massive hematemesis
 Past medical history:
 2003 – Vagotomy & pyloroplasy d/t PUD
 1996 – Dx with polycythemia vera, JAK2 positive
 Long time ago - Appendectomy
Case presentation
 Primary evaluation
 137/97, pulse 92bpm, Sat. 98%RA
 Pale
 Abdomen – soft, non-tender, enlarged spleen
 PR- normal stool
 NG – fresh blood
 Lab results: Hb-11.3 (basic-13.5), Plt-147,000 INR-1.3
Upper GI endoscopy
Triple-Phase Abdominal CT
Triple-Phase Abdominal CT
Further investigation
 AST-21
 HBV
 ALT-25
 HCV
 AP- 51
 HIV
 GGT- 45
 ANA
 Bil.- 0.6
 AMA
 Alb - 37
 ASMA
 Globulin - 17
 LKM
Liver-spleen US Elastography
Spleen Elastography
48 kPA
Liver Elastography
6.6 kPA = F1
Webb et al: Assessment of liver and spleen stiffness in patients with myelofibrosis using Fibroscan and Shear Wave
Elastography- 2015
Hematologic evaluation
 Polycythemia vera  MDS/Myelofibrosis
 CBC – normal/ near normal
 No indication for disease specific treatment
Meanwhile…
 In 6 weeks – recurrent admission
 Melena
 3rd upper endoscopy with histoacryl injection
Summary
 Recurrent bleeding from isolated gastric varices
 Huge spleen d/t MPD
 No apparent signs of liver disease
Non-cirrhotic Portal Hypertension
 Non-cirrhotic PHT –
HVPG is normal/mildly elevated and is significantly lower
than PV pressure
Non-cirrhotic portal hypertension – Diagnosis and management. Rajeev, K. et al. Journal of Hepatology 2014 vol. 60 j 421–441
Banti’s syndrome
 In 1894, G.Banti :
splenomegalic anaemia cirrhosis Banti's syndrome.
 Hypersplenism, cytopenias and portal hypertension
without portal vein obstruction or significant liver disease
 Variceal bleeding - common
 Ascites, jaundice, and encephalopathy - rare
 Idiopathic? Toxic? Immunologic?
Banti’s syndrome
 Currently AKA:
 Non-cirrhotic portal hypertension
 Idiopathic portal hypertension
 Hepatoportal sclerosis
 Non-cirrhotic portal fibrosis?
 Left-sided portal hypertension?
Polycethemia Vera & the liver
 PV - low EPO and JAK2 mutation
 Portal hypertension is found in up to 5-10% of patients
Possible mechanisms :
 Prothrombotic and hyperviscous state:
 Budd chiari syndrome / Portal vein thrombosis
 Nodular Regenerative Hyperplasia (NRH)
 Extramedullary hematopoiesis
 Splenomegaly
Marvin M. Singh, MD, Paul J. Pockros. Hematologic and Oncologic Diseases and the Liver. Clin Liver Dis 15 (2011) 69–87
Endoscopic treatment
 Timing - Patients with GI bleeding and features suggesting
cirrhosis should have upper endoscopy as soon as possible
after admission (within 12 h) (5;D)
 IGV1:
 Glue injection with N-butyl-cyanoacrylate
 Repeated at 2–3 weekly intervals until eradication
 Prophylaxis - Despite lack of data, patients with gastric
varices may be treated with NSBB (5;D).
Shunt - Angiographic
 TIPS –
HVPG < 12mmHg after TIPS prevents bleeding from
esophageal variceal but not necessarily from gastric
varices
Shunt - Surgical
 Proximal splenorenal shunt with
 Distal splenorenal shunt (DSRS)
splenectomy
End-to-side
porto-caval
shunt
Meso-caval
shunt
Spleen reduction in MPD
 Splenic artery embolization
 Radiotherapy
 Splenectomy
Ruxolitinib (Jakavi)
 The first JAK inhibitor to gain approval
 In randomized Phase III trials - Reduction in spleen volume (>35%):
 at 24 weeks in 41.9% vs 0.7% placebo; P<0.0001
 ~70% maintained their response for 48 weeks
 AEs - anemia and thrombocytopenia
 Responses to ruxolitinib are typically observed within the first 3–6
months after therapy initiation
Back to our patient…
Following multidisciplinary discussions:
 Plan A! – Jakavi
If fails…
 Plan B – Liver biopsy & HVPG 
TIPS/splenectomy/other?