Ishida Y, Utikoshi M, Kurosaki M, Ohta K, Chujo T, Aoyama S, Ohsawa K, Saito K, Yokoyama H, Ohta S
Department of Internal Medicine, Toyama City Hospital, Toyama.
Intern Med. 1998 Aug;37(8):694-9. doi: 10.2169/internalmedicine.37.694.
A 50-year-old woman was treated with prednisolone for polymyositis. During the therapy, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) occurred. Neither plasma infusion nor plasma exchange could relieve the clinical manifestations of TTP/HUS. Moreover, massive ascites appeared and worsened her condition. She died approximately one year after the diagnosis of polymyositis. The autopsy revealed centri-lobular hepatic necrosis and nonthrombotic obliteration of hepatic small veins. The diagnosis of hepatic veno-occlusive disease (VOD) was made. It was suspected that common factors other than cytoreductive therapy had damaged the endothelium and caused TTP/HUS and VOD in a case of polymyositis.
一名50岁女性因多发性肌炎接受泼尼松龙治疗。治疗期间，发生了血栓性血小板减少性紫癜/溶血性尿毒症综合征（TTP/HUS）。血浆输注和血浆置换均无法缓解TTP/HUS的临床表现。此外，出现大量腹水，病情恶化。她在诊断为多发性肌炎后约一年死亡。尸检显示小叶中心性肝坏死和肝小静脉非血栓性闭塞。诊断为肝静脉闭塞病（VOD）。怀疑在一例多发性肌炎中，除细胞减灭治疗外的共同因素损伤了内皮，导致了TTP/HUS和VOD。
