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血栓性血小板减少性紫癜并发系统性红斑狼疮。血浆置换时代的病例报告及文献综述。

Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus. Case report and literature review from the plasmapheresis era.

作者信息

Stricker R B, Davis J A, Gershow J, Yamamoto K S, Kiprov D D

机构信息

Department of Medicine, California Pacific Medical Center, San Francisco 94120.

出版信息

J Rheumatol. 1992 Sep;19(9):1469-73.

PMID:1433019
Abstract

Although thrombotic thrombocytopenic purpura (TTP) has been described in patients with systemic lupus erythematosus (SLE), the relationship between these 2 diseases is controversial. We recently treated a patient with longstanding SLE who developed TTP. The patient responded to therapy with aggressive plasmapheresis. Review of the literature revealed that TTP may occur in the setting of either active or inactive SLE. Survival in SLE associated TTP correlated with the use of plasma therapy (plasma infusion or plasmapheresis) rather than with the activity of the underlying autoimmune disease. We conclude that TTP and SLE represent distinct clinical entities that may occur together in an immunologically predisposed host. The use of plasma therapy appears to have had a significant impact on survival in these patients.

摘要

尽管血栓性血小板减少性紫癜(TTP)已在系统性红斑狼疮(SLE)患者中被描述,但这两种疾病之间的关系仍存在争议。我们最近治疗了一名患有长期SLE且发生TTP的患者。该患者对积极的血浆置换治疗有反应。文献回顾显示,TTP可能发生在活动性或非活动性SLE的情况下。SLE相关TTP的生存率与血浆治疗(血浆输注或血浆置换)的使用相关,而非与潜在自身免疫性疾病的活动度相关。我们得出结论,TTP和SLE代表不同的临床实体,可能在免疫易感宿主中共同出现。血浆治疗的使用似乎对这些患者的生存率有显著影响。

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