Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey.
Transfusion. 2013 Jul;53(7):1586-93. doi: 10.1111/j.1537-2995.2012.03944.x. Epub 2012 Nov 1.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurologic disturbances, and renal failure. Plasma therapy has dramatically improved prognosis of TTP, whereas recurrent acute episodes still occur in approximately 40% of patients. Moreover, patients with acquired ADAMTS13 deficiency, which is a significant factor for relapse, may require additional immunosuppressive treatment to get a durable remission.
We hereby report two patients with a history of relapsed idiopathic TTP, who both received cyclosporin A (CSA) as a prophylactic manner after the remission was achieved. We also discuss the efficacy of CSA in patients with relapsed idiopathic TTP with a review of the published literature.
Under CSA therapy, both patients maintained their clinical remission state, and the ADAMTS13 levels were normalized.
To conclude, CSA therapy may be useful for the prevention of relapsed idiopathic TTP in patients with a history of frequent relapses.
血栓性血小板减少性紫癜（TTP）是一种危及生命的疾病，其特征为微血管性溶血性贫血、血小板减少、发热、神经功能紊乱和肾衰竭。血浆治疗显著改善了 TTP 的预后，但约 40%的患者仍会出现复发性急性发作。此外，患有获得性 ADAMTS13 缺乏症（复发的重要因素）的患者可能需要额外的免疫抑制治疗以获得持久缓解。
我们报告了两名复发性特发性 TTP 患者的病史，他们在缓解后均以环孢素 A（CSA）作为预防措施。我们还讨论了 CSA 在复发性特发性 TTP 患者中的疗效，并回顾了已发表的文献。
在 CSA 治疗下，两名患者均保持临床缓解状态，ADAMTS13 水平正常。
总之，CSA 治疗可能对有频繁复发史的特发性 TTP 患者的复发预防有用。
