Hattori A, Tatewaki W
Sado General Hospital.
Nihon Rinsho. 1993 Jan;51(1):178-83.
Thrombotic thrombocytopenic purpura (TTP), uncommon and still unclarified in pathogenesis, has been treated by various methods, including antiplatelet drugs, steroids, heparin and recently, plasma infusion, and plasma exchange. To evaluate the effectiveness of each therapy or its combination, we analyzed the data of 56 patients with TTP in Japan by a questionnaire. The mortality was 26.8%. Plasma exchange and infusion usually combined with other drugs (steroids, antiplatelet drugs etc.) seemed more effective than therapies without exchange or infusion. We employed a scoring method to express the severity of TTP, using platelet count, hemoglobin level and serum lactate dehydrogenase, creatinine and bilirubin levels as parameters. This scoring method showed objectively the effectiveness of antiplatelet drugs, steroids, or plasma exchange or infusion, and especially, the effectiveness of the latter two (singly or combined) in seven patients who did not respond to other therapies. In conclusion, plasma exchange and infusion are suggested for the treatment of TTP, especially, the severe type. Combination of antiplatelet drugs and/or steroids remains to be evaluated.
血栓性血小板减少性紫癜（TTP）较为罕见，发病机制仍不明确，其治疗方法多种多样，包括抗血小板药物、类固醇、肝素，以及近来采用的血浆输注和血浆置换。为评估每种治疗方法或其联合治疗的效果，我们通过问卷调查分析了日本56例TTP患者的数据。死亡率为26.8%。血浆置换和输注通常与其他药物（类固醇、抗血小板药物等）联合使用，似乎比未进行置换或输注的治疗方法更有效。我们采用评分方法来表示TTP的严重程度，以血小板计数、血红蛋白水平、血清乳酸脱氢酶、肌酐和胆红素水平作为参数。该评分方法客观地显示了抗血小板药物、类固醇、血浆置换或输注的效果，尤其是后两者（单独或联合使用）对7例对其他治疗无反应患者的效果。总之，建议采用血浆置换和输注治疗TTP，尤其是重症类型。抗血小板药物和/或类固醇的联合使用仍有待评估。
