Budd-Chiari syndrome and myeloproliferative disorder.

Budd-Chiari syndrome (BCS) is a rare clinicopathological condition characterized by occlusion of the major hepatic veins with or without membranous obstruction (MOVC)of the inferior vena cava (IVC) at its hepatic portion. Ascites, hepatomegaly and upper abdominal pain constitute the classical triad of symptoms, and the disease usually progresses to congenital liver cirrhosis and portal hypertension ( 1 ). Most of the Japanese cases of BCS are idiopathic with MOVC,and no associated etiology is demonstrable, whereas in Western countries, most patients have underlying thrombogenic conditions, such as myeloproliferative disorders (MPD), paroxysmal nocturnal