Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus
Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus
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DOI：
10.1159/000089469
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发表时间：
2006-01-01
影响因子：
2.4
通讯作者：
Al-Sugair, S
中科院分区：
文献类型：
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作者：
Aleem, A;Al-Sugair, S
Karger AG，巴塞尔。血栓性血小板减少性紫癜（TTP）发生在系统性红斑狼疮（SLE）患者中是罕见的，并且由于两种疾病的重叠特征而难以诊断。本研究的目的是进一步描述这种不常见的相关性的表现特征，诊断困难和治疗结果。这是来自一个单一中心的最大系列，在6年的时间内诊断出6例患者。三分之二的患者同时诊断为TTP和SLE。半数患者Coombs试验阳性，沿着有明确的TTP特征。5例患者接受血浆置换作为初始治疗，1例患者仅接受血浆输注。5例患者中有4例对血浆置换术有反应，只有1例患者需要细胞毒性治疗。与SLE相关的TTP似乎诊断不足，在这种情况下，阳性Coombs试验并不反对TTP的诊断。大多数病人对血浆置换反应良好。如果反应不佳，应尽早考虑使用细胞毒性药物。版权所有（C）2006 S.
Karger AG, Basel. Thrombotic thrombocytopenic purpura (TTP) occurring in patients with systemic lupus erythematosus (SLE) is rare and can be difficult to diagnose because of overlapping features of the two disorders. The aim of this study is to further characterize this uncommon association in terms of presenting features, diagnostic difficulties and treatment outcome. This is the largest series from a single centre with 6 patients diagnosed over a 6-year period. Two thirds of the patients had a simultaneous diagnosis of TTP and SLE. Half of the patients had a positive Coombs test along with clear features of TTP. Five patients received plasmapheresis as initial treatment while 1 patient received plasma infusions only. Four out of 5 patients responded to plasmapheresis and only 1 patient required cytotoxic therapy. TTP in association with SLE appears to be underdiagnosed and a positive Coombs test is not against the diagnosis of TTP in this setting. Most of the patients respond well to plasmapheresis. In case of a poor response, cytotoxic drugs should be considered early. Copyright (C) 2006 S.