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目的:探讨系统性红斑狼疮(SLE)相关性血栓性血小板减少性紫癜(TTP)的临床特征、诊断、治疗及转归。方法:回顾性分析河北医科大学第二医院2002年1月至2015年8月收治的12例SLE相关性TTP患者的临床资料。结果:12例SLE相关性TTP患者包括女11例,男1例,中位年龄34.5岁,其中5例为SLE治疗过程中出现的TTP,7例为两者同时诊断;全部患者均有溶血性贫血,血小板减少,神经系统损害,10例有肾脏损害;5例血管性血友病因子裂解蛋白酶(ADAMTS 13)活性均重度减低(<5%),同时伴抑制物阳性;9例外周血涂片破碎红细胞均>1%;12例患者均给予糖皮质激素治疗,11例联合其他药物(10例联合免疫抑制剂,1例联合静脉注射用丙种球蛋白,1例同时加用美罗华);10例给予血浆置换,2例死亡;2例未行血浆置换患者均死亡;4例死亡患者全部伴有肾脏损害。结论:临床表现结合多次血涂片检查有助于SLE相关性TTP的早期诊断,尽早血浆置换联合糖皮质激素是治疗成功的重要措施,肾脏损害可能提示预后不良。
Objective: To investigate the clinical features, diagnosis, treatment and prognosis of systemic lupus erythematosus (SLE) -related thrombotic thrombocytopenic purpura (TTP). Methods: The clinical data of 12 SLE-related TTP patients admitted to the Second Hospital of Hebei Medical University from January 2002 to August 2015 were retrospectively analyzed. Results: Twelve SLE-related TTP patients including 11 females, 1 males, the median age of 34.5 years, of which 5 cases of SLE TTP occurred in the course of treatment, 7 cases were both diagnosed; all patients were hemolytic Anemia, thrombocytopenia and nervous system damage, and 10 cases of renal damage. Five cases of ADAMTS 13 activity were significantly reduced (<5%), accompanied by positive inhibitors; nine cases of peripheral blood smear All the 12 cases were treated with glucocorticoid, 11 cases were combined with other drugs (10 combined immunosuppressive agents, 1 with intravenous gamma globulin and 1 with rituximab); 10 Cases were given plasma exchange, 2 patients died; 2 patients died without plasma exchange; 4 patients died of renal damage. Conclusion: The clinical manifestations combined with multiple blood smears are helpful for the early diagnosis of SLE-associated TTP. As soon as possible, plasma exchange combined with glucocorticoid is an important measure for successful treatment. Kidney damage may indicate poor prognosis.