Ruggenenti P, Remuzzi G
Ospedali Riuniti di Bergamo, Italy.
Hematol Oncol Clin North Am. 1990 Feb;4(1):219-41.
This article provides us with background information on the disease. Clinical features, variants and classification, laboratory findings, and pathology are discussed. Knowledge of the disease's pathogenesis has increased recently and specific causes discussed are predisposing factors, triggering agents, endothelial damage, defective PGI2 bioavailability, FVIII/vWF multimeric structure abnormalities, platelet activation, and hemolytic anemia. Proposed specific therapies discussed are steroids, heparin, antiplatelet agents, prostacyclin, splenectomy, immunosuppressive agents, plasma infusion, and plasma exchange.
本文为我们提供了该疾病的背景信息。文中讨论了临床特征、变异与分类、实验室检查结果以及病理学。近年来，对该疾病发病机制的认识有所增加，所讨论的具体病因包括易感因素、触发因子、内皮损伤、前列环素（PGI2）生物利用度缺陷、FVIII/vWF多聚体结构异常、血小板活化以及溶血性贫血。所讨论的拟用特异性治疗方法包括类固醇、肝素、抗血小板药物、前列环素、脾切除术、免疫抑制剂、血浆输注和血浆置换。
