Ryan P F, Cooper I A, Firkin B G
Med J Aust. 1979 Feb 10;1(3):69-72. doi: 10.5694/j.1326-5377.1979.tb112002.x.
Thrombotic thrombocytopenic purpura, an acute clinical disorder of uncertain aetiology, has previously been associated with almost 100% mortality. Dramatic response to exchange transfusion has been reported, and there is some evidence that a circulating immune complex may be involved in the pathogenesis. Therefore, it was decided to employ large-volume plasma exchange by means of a continuous-flow cell separator in the management of five patients with this disease. Three patients showed a dramatic response, which was manifested by rapid correction of the neurological deficit and of the abnormalities in laboratory findings. We conclude that the thrombotic thrombocytopenic syndrome should be regarded as an indication for emergency plasma exchange.
血栓性血小板减少性紫癜是一种病因不明的急性临床病症,以往其死亡率几乎达100%。有报告称换血疗法对此病疗效显著,且有证据表明循环免疫复合物可能参与其发病机制。因此,决定采用连续流动细胞分离器对5例该病患者进行大容量血浆置换治疗。3例患者疗效显著,表现为神经功能缺损及实验室检查异常迅速得到纠正。我们得出结论,血栓性血小板减少综合征应被视为紧急血浆置换的适应证。