Non-cirrhotic Portal Hypertension: Think of Chronic Myelomonocytic Leukemia — A Case Report

Abstract

Non-cirrhotic portal hypertension is an uncommon cause of variceal bleeding and requires careful evaluation for vascular, hepatic, infectious, autoimmune, metabolic, and haematological causes. Chronic myelomonocytic leukaemia is a clonal haematological malignancy characterised by persistent monocytosis, bone marrow dysplasia, and a variable risk of progression to acute myeloid leukaemia. A hepatic or portal hypertensive presentation is unusual. We report the case of a 68-year-old man with no known history of liver disease who presented with massive haematemesis associated with progressive asthenia and long-standing abdominal heaviness. Clinical examination revealed pallor, massive splenomegaly extending beyond the umbilicus, and moderate hepatomegaly, without signs of hepatocellular insufficiency or stigmata of cirrhosis. Upper gastrointestinal endoscopy showed grade II oesophageal varices with red signs, treated by endoscopic band ligation. Abdominal ultrasound confirmed splenomegaly and portal vein dilatation, while Doppler ultrasound showed a patent portal trunk without thrombosis. Liver elastography did not show advanced fibrosis, and hepatitis B and C serologies were negative. Laboratory tests revealed leukocytosis at 32 × 10⁹/L, persistent monocytosis at 4.5 × 10⁹/L, normocytic anaemia at 9 g/dL, and thrombocytopenia at 95 × 10⁹/L. Bone marrow aspiration demonstrated hypercellularity, myeloid dysplasia, monocytic proliferation, and blasts below 5%, supporting the diagnosis of chronic myelomonocytic leukaemia. The patient received non-selective beta-blockers and hydroxyurea, with haematological stabilisation and no recurrent bleeding at six months.