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作 者:李旭 刘方久[1] 杨洪 LI Xu;LIU Fangjiu;YANG Hong(Suining Central Hospital,Suining 629000,China)
出 处:《中国输血杂志》2024年第10期1189-1192,共4页Chinese Journal of Blood Transfusion
摘 要:目的分析讨论1例以血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)为首发表现的患者,在多种自身抗体异常情况下考虑为系统性红斑狼疮(Systemic lupus erythematosus,SLE),通过血浆置换联合激素冲击治疗后的临床结局,总结分享治疗经验。方法1例中年女性初始表现为发热、出血、溶血血小板减少,实验室检查提示血管性血友病因子裂解酶13(thrombospondin type 1 motif no.13,ADAMTS13)活性降低,自身抗体异常,诊断考虑SLE合并TTP,进行连续多次血浆置换联合大剂量激素冲击治疗,动态观察患者血小板恢复情况。结果患者对血浆置换联合激素冲击治疗反应良好,血小板在短时间内恢复至正常值,激素逐渐减量,在随访期间并未出现复发情况。结论TTP合并狼疮并不多见,两种疾病同时发生时血浆置换联合激素冲击治疗可能有助于病情快速缓解,该治疗方案可为临床类似病例治疗提供参考。Objective To analyze the clinical outcomes of a patient with thrombotic thrombocytopenic purpura(TTP)as the starting performance who was considered as systemic lupus erythematosus(SLE)under multiple abnormal autoantibodies,and summarize the treatment experience through the clinical outcomes of plasma exchange combined with high-does steroid pulse therapy.Methods A middle-aged female patient presented with initial symptoms of fever,bleeding and hemolytic thrombocytopenia.Laboratory tests showed decreased activity of thrombospondin type 1 motif no.13(ADAMT13)and abnormal autoantibodies.The diagnosis was considered SLE with TTP,and the patient underwent continuous plasma exchange combined with high-dose steroid pulse therapy.The patient′s platelet recovery was dynamically observed.Results The patient responded well to plasma exchange combined with high-does steroid pulse therapy,with platelet count raising to normal in a short period of time and hormone gradually decreasing.There was no recurrence during the follow-up period.Conclusion TTP combined with lupus is not common,but when both occur simultaneously,plasma exchange combined with high-does steroid pulse therapy may effectively alleviate symptoms.This treatment approach can serve as a valuable reference for similar clinical cases.
关 键 词:血浆置换 激素冲击 系统性红斑狼疮 血栓性血小板减少性紫癜
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