Acquired thrombotic thrombocytopenic purpura as the presenting symptom of systemic lupus erythematosus. Successful treatment with plasma exchange and immunosuppression - report of two cases
Acquired thrombotic thrombocytopenic purpura as the presenting symptom of systemic lupus erythematosus. Successful treatment with plasma exchange and immunosuppression - report of two cases
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DOI：
10.1111/j.1600-0609.2005.00526.x
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发表时间：
2005-11-01
影响因子：
3.1
通讯作者：
Nerl, C
中科院分区：
文献类型：
--
作者：
Starck, M;Abedinpour, F;Nerl, C
血栓性血小板减少性紫癜（TTP）是一种罕见但危及生命的综合征，其特征是血小板聚集引起闭塞性微血管病变。它被描述为系统性红斑狼疮（SLE）的并发症。最近的研究表明，遗传或自身抗体诱导的金属蛋白酶ADAMTS13缺乏在TTP的发病机制中起关键作用。这里我们报告两例罕见的TTP作为SLE的首发症状。两例患者均经血浆置换联合免疫抑制治疗，均完全康复。虽然TTP和SLE有一些共同的临床表现，而且这两种疾病共存的频率可能比我们目前认为的要高，但一种疾病的特征不应该误导我们拒绝另一种疾病。
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening syndrome characterized by platelet aggregation causing occlusive microangiopathy. It has been described as a complication in systemic lupus erythematosus (SLE). Recent research indicated that genetic or autoantibody-induced deficiency of the metalloprotease ADAMTS13 plays a key role in the pathogenesis of TTP. Here we report two uncommon cases of TTP as the first presenting symptom of SLE. Both patients were treated with combined plasma exchange and immunosuppressive therapy, and recovered completely. Although TTP and SLE have several clinical findings in common, and both disorders may coexist more frequently than we currently assume, features of one disease should not mislead to reject the alternative disorder.