Hepatic veno-occlusive disease.

Hepatic veno-occlusive disease is a nonthrombotic obliteration of small intrahepatic veins by loose connective tissue. The venous occlusion may be progressive and lead to massive hepatocellular necrosis. Although originally described as a result of intoxication with plant alkaloids, it is now seen as a complication of high-dose antineoplastic chemotherapy, especially in the setting of bone marrow transplantation. The incidence of hepatic veno-occlusive disease after bone marrow transplantation approaches 20 percent, with mortality ranging from 7 to 50 percent. The clinical diagnosis may be quite accurate (as confirmed by biopsy or autopsy) and is based on the triad of jaundice, hepatomegaly and/or right upper quadrant pain, and ascites or unexplained weight gain. The pathogenesis is obscure but most likely relates to drug-induced venous endothelial damage. At the present time, therapy for veno-occlusive disease of the liver remains supportive. As intensive chemotherapy regimens (with or without bone marrow support) become more widely applied, it is expected that this disease will be encountered more frequently.