2970 Non-Cirrhotic Portal Hypertension in Myeloproliferative Disease: Left and Right-Sided Component of Portal Hypertension

Abstract

INTRODUCTION: NCPH is the presence of portal hypertension (PH) in the absence of cirrhosis. While Schistosomiasis is the commonest cause in West, myeloproliferative diseases (MPD) characterized by cellular proliferation of one or more hematologic cell lines resulting in extra-medullary hematopoiesis predominantly in the spleen and occasionally in liver may cause PH. Pathogenesis of NCPH in MPD revolves around the concept of left sided portal hypertension. This study presents a cohort of Indian patients of NCPH with MPD. METHODS: 260 patients with NCPH were evaluated and 9 MPD was diagnosed based on 2008 WHO-criteria. Cirrhosis was ruled out by fibroscan and liver biopsy. All patients underwent a pro coagulant screen which included protein-C, protein-S, factor-V leiden, antithrombin D, anti phospholipid antibody, prothrombin gene deficiency and Jak-2 mutation study. Routine PH evaluation and Fibroscan and liver biopsy was done in all cases. RESULTS: Out of 9 patients 4 (44.4%) each had polycythemia-rubra-vera (PRV) and myelofibrosis while 1 had essential thrombocytosis. Median age was 50 ± 7 yrs with 8 male and 1 female patient. 5 (55.5%) presented with symptomatic splenomegaly, 1 (11.1%) with ascites and 3 (33%) with GI bleed and comprised of 64.3% in PRV. Splenomegaly was universally present and hepatomegaly in 44.4%. Isolated gastric varices was present in 3 and gastroesophageal varices in 4 and isolated esophageal varices in 2 patients. Except for Jak-2 mutation found positive in 100% cases of PRV and 66.7% cases of MF, none of the other markers were positive. None, except 1 patient on liver biopsy showed sinusoidal deposits. 8/9 patients underwent endoscopic therapy for varices and were given prophylaxis with carvedilol. Mean follow up was 40 months and none had recurrent bleed or worsening of liver function due to PH. CONCLUSION: True incidence MPD causing PH is under-recognised. Our results are similar to other studies from Asia. Unlike other studies, we did not find portal vein thrombosis. MPD patients had huge splenomegaly leading to increased blood flow through the splenic vein leading to left sided portal hypertension. Myeloid metaplasia in liver can also leading to right-sided PH. Whether beta blockers would help as primary or secondary prophylaxis without this component is inconclusive. However, in our cohort of patient they were helpful. Rescue therapy like splenectomy, JAK 2 inhibitors, TIPS etc may be reserved for the refractory cases.