Chronic relapsing thrombotic thrombocytopenic purpura: Role of therapy with cyclosporine
Chronic relapsing thrombotic thrombocytopenic purpura: Role of therapy with cyclosporine
复制标题
慢性复发性血栓性血小板减少性紫癜：环孢素治疗的作用
DOI：
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
G. Chikkappa
中科院分区：
文献类型：
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作者：
D. Pasquale;Rakesh Vidhya;K. DaSilva;M. Tsan;L. Lansing;G. Chikkappa
复发性血栓性血小板减少性紫癜（TTP）是一种罕见的疾病，大多数人经历1至5次复发。我们报告一个病人有18次血栓性血小板减少性紫癜（TTP），迄今为止最高的复发次数。最后11次发作用含有环孢霉素的方案治疗。对患者的病历进行了审查，以获得相关的临床、实验室和治疗数据。我们总结了每次发作的各种参数，并比较了用与不用环孢素治疗的复发特征。TTP的初始发作是不寻常的，因为它没有回应血浆置换，糖皮质激素，新鲜冷冻血浆（FFP）。仅在脾切除术后缓解。Epilepsy 2-7对FFP加泼尼松有反应。第8次发作对泼尼松加FFP无效，但用环孢素加泼尼松后迅速缓解。随后，2例复发对环孢素单药治疗有效，2例对环孢素+FFP治疗有效，4例对环孢素+泼尼松± FFP治疗有效，2例对环孢素、FFP、泼尼松和血浆置换治疗有效。在缓解持续时间，或复发的严重程度或持续时间方面，用与不用环孢霉素治疗没有差异。然而，环孢霉素的使用显著降低了对泼尼松的需求和维持治疗的时间;因此，它主要作为TTP的连续治疗有效。Am.血液学杂志57：57-61，1998.出版于1998年Wiley利斯公司本条目属于美国政府作品，因此在美国属于公有领域。
Relapsing thrombotic thrombocytopenic purpura (TTP) is a rare disorder with most individuals experiencing 1 to 5 relapses. We report a patient with 18 episodes of thrombotic thrombocytopenic purpura (TTP), the highest number of relapses thus far described. The last 11 episodes were treated with regimens containing cyclosporine. The patient's medical record was reviewed for pertinent clinical, laboratory, and treatment data. We summarized various parameters for each episode and compared characteristics of relapses treated with vs. without cyclosporine. The initial episode of TTP was unusual in that it failed to respond to plasmapheresis, glucocorticoids, and fresh frozen plasma (FFP). It remitted only following splenectomy. Episodes 2–7 responded to FFP plus prednisone. Episode 8 failed to respond to prednisone plus FFP but remitted promptly with cyclosporine plus prednisone. Subsequently, 2 relapses responded to cyclosporine alone, 2 to cyclosporine plus FFP, 4 to cyclosporine plus prednisone ± FFP, and 2 to cyclosporine, FFP, prednisone, and plasma exchange. There was no difference in remission duration, or in severity or duration of relapses treated with vs. without cyclosporine. Use of cyclosporine, however, significantly decreased the requirement for prednisone and the length of maintenance therapy; thus it is effective mainly as an adjunctive therapy for TTP. Am. J. Hematol. 57:57–61, 1998. Published 1998 Wiley‐Liss, Inc. This article is a U.S. Government work and, as such, is in the public domain in the United States of America.
影响因子：
20.3
作者：
C. Dinarello
通讯作者：
C. Dinarello