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首页> 外文期刊>Journal of pediatric hematology/oncology: Official journal of the American Society of Pediatric Hematology/Oncology >Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus.
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Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus.

机译：小儿系统性红斑狼疮的血栓性血小板减少性紫癜。

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摘要

We report a child with thrombotic thrombocytopenic purpura (TTP) secondary to systemic lupus erythematosus. The diagnosis was confirmed by low ADAMTS13 activity (<5%) along with the presence of a low titer inhibitor. Her clinical course was complicated by systemic lupus erythematosus, immunosuppressant therapy, and septic shock. She responded to plasma exchange and ADAMTS13 activity levels recovered. This case illustrates the heterogeneity of TTP and the difficulty of making a diagnosis of TTP. ADAMTS13 activity assay can be useful in the differential diagnosis of diseases with clinical features of thrombotic microangiopathy in pediatric patients. However, treatment needs to be decided carefully case-by-case.
机译：我们报告儿童继发于系统性红斑狼疮的血栓性血小板减少性紫癜（TTP）。低ADAMTS13活性（<5％）以及低滴度抑制剂的存在证实了该诊断。她的临床过程因全身性红斑狼疮，免疫抑制剂治疗和败血性休克而复杂化。她对血浆交换做出了反应，ADAMTS13活性水平恢复。这种情况说明了TTP的异质性和诊断TTP的困难。 ADAMTS13活性测定可用于对儿科患者具有血栓性微血管病临床特征的疾病进行鉴别诊断。但是，治疗需要根据具体情况仔细决定。

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