Wereport a 24-year-old Japanese female hospitalized withjaundice and ascites. She exhibited hepatosplenomegaly, severe liver dysfunction, and slight polycythemia with an increase in serum levels of p-thromboglobin and platelet factor 4. Bone marrow was hypercellular with an increase in progenitor cells. The aggregation response of platelets to ADPand to collagen was markedly increased. Venography revealed narrowed hepatic veins with 'spider web' sign. Liver biopsy revealed hepatic congestion. Budd-Chiari synd…
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