BUDD-CHIARI SYNDROME CAUSED BY HEPATIC VEIN-THROMBOSIS IN A PATIENT WITH MYELOPROLIFERATIVE DISORDER

We report a 24-year-old Japanese female hospitalized with jaundice and ascites. She exhibited hepatosplenomegaly, severe liver dysfunction, and slight polycythemia with an increase in serum levels of beta-throm boglobin and platelet factor 4. Bone marrow was hypercellular with an increase in progenitor cells. The aggregation response of platelets to ADP and to collagen was markedly increased. Venography revealed narro wed hepatic veins with 'spider web' sign. Liver biopsy revealed hepati c congestion. Budd-Chiari syndrome was diagnosed, and was thought to b e due to thrombosis related to myeloproliferative disorder. Liver tran splant was successful in relieving symptoms.