HEPATIC VENOOCCLUSIVE DISEASE IN A CASE OF POLYMYOSITIS ASSOCIATED WITH THROMBOTIC THROMBOCYTOPENIC PURPURA HEMOLYTIC-UREMIC SYNDROME

A 50-year-old woman was treated with prednisolone for polymyositis. Du ring the therapy, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) occurred. Neither plasma infusion nor plasma excha nge could relieve the clinical manifestations of TTP/HUS, Moreover, ma ssive ascites appeared and worsened her condition. She died approximat ely one year after the diagnosis of polymyositis. The autopsy revealed centri-lobular hepatic necrosis and nonthrombotic obliteration of hep atic small veins. The diagnosis of hepatic veno-occlusive disease (VOD ) was made. It was suspected that common factors other than cytoreduct ive therapy had damaged the endothelium and caused TTP/HUS and VOD in a case of polymyositis.