Essential thrombocythemia in Hepatitis B-related cirrhosis with esophageal varices: a rare case report

Abstract

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm driven by clonal megakaryocytic proliferation, presenting with sustained thrombocytosis and an increased risk of thrombotic and hemorrhagic events. In contrast, decompensated cirrhosis secondary to chronic hepatitis B is more commonly associated with thrombocytopenia, due to hypersplenism and impaired thrombopoietin production. Their coexistence is rare and poses diagnostic and therapeutic challenges. We present a 63-year-old male with chronic hepatitis B who developed recurrent melena, fatigue, and signs of portal hypertension, including splenomegaly, mild ascites, and grade III esophageal varices. Laboratory findings revealed microcytic hypochromic anemia (Hb 9.2 g/dL, MCV 74.8 fL), hypoalbuminemia (2.9 g/dL), and paradoxical extreme thrombocytosis (>1,500 × 10⁹/L). Fibroscan demonstrated advanced cirrhosis (20 kPa), bone marrow aspirate showed hyperlobulated megakaryocytes with platelet clusters, and molecular testing confirmed JAK2 V617F mutation, consistent with ET. Management included propranolol, spironolactone, endoscopic variceal ligation, tenofovir, proton
pump inhibitors, and intravenous iron. Aspirin was withheld, given the risk of variceal bleeding, while cytoreductive therapy was not available under national insurance coverage. This case illustrates the importance of considering alternative hematologic diagnoses in cirrhotic patients when laboratory abnormalities deviate from expected patterns. The recognition of JAK2-positive ET in cirrhosis underscores the diagnostic utility of
molecular testing and the necessity of a multidisciplinary approach. Clinical management must carefully balance the opposing risks of thrombosis and variceal bleeding. Furthermore, limitations in access to cytoreductive therapy in resource-constrained settings highlight the urgent need for individualized
strategies and healthcare policy advocacy to optimize outcomes in such rare dual-pathology scenarios.