目的　探讨系统性红斑狼疮（SLE）合并血栓性血小板减少性紫癜（TTP）的临床特点、诊断和治疗。方法 回顾分析1例重症SLE合并TTP患儿的临床资料，并复习相关文献。结果　患儿，女，13岁，以肾病综合征起病，诊断重症 SLE、狼疮性肾炎。初治好转后再现头痛、血小板减少、贫血、急性肾损伤及发热。血涂片破碎红细胞2%，诊断合并TTP。经 激素冲击、血浆置换、免疫抑制剂等综合性治疗后病情缓解。结论　SLE合并TTP较为少见，病死率高，早期识别、合理治 疗可改善预后。

Objective　To explore the clinical characteristics, diagnosis and treatment of systemic lupus erythematosus (SLE) with thrombotic thrombocytopenic purpura (TTP). Method　The clinical data of SLE combined with TTP in a child were analyzed retrospectively, and the related literature was reviewed. Results　A 13-year-old girl who was presented with onset of nephrotic syndrome was diagnosed with severe SLE and lupus nephritis. Headache, thrombocytopenia, anemia, acute kidney injury and fever reappeared after initial treatment and improvement. The ratio of broken red blood cells in the blood smear was 2%, and TTP was diagnosed. After comprehensive treatment such as high dose of hormones, plasma exchange, and application of immunosuppressive agents, her condition was relieved. Conclusion　SLE with TTP is relatively rare and has a high mortality. Early recognition and appropriate treatment can improve the prognosis.