[A case of cavernoma of portal vein associated with polycythemia vera]

Cavernoma of the portal vein is defined as a formation of venous channels within or around a previously thrombosed portal vein. We experienced a 50-year-old woman who presented a huge hepatic mass with right upper quadrant dull pain. Abdominal computed tomography showed a huge sponge-like hepatic mass with cavernous transformation of portal vein along the common bile duct and common hepatic duct. She had increased hemoglobin/hematocrit (15.7 g/dL/49.1%) and red blood cell mass (35 mL/kg). Platelet count was 450,000/microL and white blood cell count was 13,500/microL. Erythropoietin level was low normal range (10.2 mU/mL). Bone marrow biopsy showed a moderately hypercellular marrow and overall cellularity was about 80-90%. Megakaryocytes were slightly increased in number with abnormal clusterings Myelopoiesis and erythropoiesis were also slightly increased with moderate to severe fibrosis. She was diagnosed as polycythemia vera with cavernous transformation of portal vein. Repeated thrombosis occurred in the leg and the toe and was treated with angioplasty and thrombolytic therapy with phlebotomy.