获得性血栓性血小板减少性紫癜16例临床分析

摘要

Objective To investigate the clinical manifestations,laboratory characteristics,treatment strategies and outcome of patients with acquired thrombotic thrombocytopenic purpura(TTP). Methods A total of 16 acquired TTP patients admitted to the Second Hospital of Hebei Medical University from January 2013 to December 2014 were enrolled in the study. A retrospective analysis of clinical manifestations,laboratory examination data,ADAMTS 13 activity,inhibitor test results, treatment method and efficacy was conducted. Results Five(31. 3% )patients had typical " Reynolds' pentad" of the 16 patients,all the patients had different degrees of microangiopathic hemolytic anemia and thrombocytopenia. 14 patients received peripheral blood smear examination,and broken red blood cells were found in 10 patients, with 6 > 1% . The lactate dehydrogenase(LDH)of 15 patients was increased,and the indirect bilirubin(IBIL)of 16 patients was increased. ADAMTS 13 activity reduced in all patients,with 15 patient less than 10% ;the results of ADAMTS 13 inhibitor test were all positive. Among the 16 patients,2 patients died before treatment,1 patient quitted treatment,the rest 13 patients were administrated with PE combined with corticosteroid,among which 2 patients died within 24 hours after treatment began and 11 patients saw the treatment effective. Conclusion The diagnosis of acquired TTP is based on the presenting clinical features: thrombocytopenia, microangiopathic hemolytic anemia,neurologic abnormalities,renal failure and fever. A severe decrease of ADAMTS 13 activity with the presence of an inhibitor confirms the diagnosis of acquired TTP. Early administration of PE combined with immunosuppressors is the essential element for the effective emergency treatment of acquired TTP.%目的：总结获得性血栓性血小板减少性紫癜（TTP）患者的临床表现、实验室检查、治疗及转归。方法选取2013年1月—2014年12月河北医科大学第二医院收治的所有获得性 TTP 患者16例为研究对象。回顾性分析患者的临床表现、实验室检查资料、血浆血管性血友病因子裂解蛋白酶（ADAMTS 13）活性及其抑制物检查结果、治疗方法及疗效。结果16例患者中，5例（31.3％）有典型的“五联征”。所有患者有不同程度的微血管病性溶血性贫血和血小板计数（PLT）减少。14例患者行外周血涂片检查，10例可见破碎红细胞，6例破碎红细胞＞1％。15例患者乳酸脱氢酶（LDH）升高，所有患者间接胆红素（DBIL）升高。16例患者 ADAMTS 13活性均减低，其中15例ADAMTS 13活性＜10％；ADAMTS 13抑制物检测结果均为阳性。16例患者中，2例于治疗前死亡，1例放弃治疗，其余13例给予血浆置换（PE）联合糖皮质激素治疗，其中2例于治疗开始24 h 内死亡，11例患者治疗有效。结论获得性 TTP 诊断主要依靠临床特征，包括微血管病性溶血性贫血、PLT 减少、神经系统异常、肾功能异常、发热， ADAMTS 13活性重度减低及其抑制物阳性是诊断获得性 TTP 的重要实验室依据，尽早开始 PE 治疗，同时联合免疫抑制剂治疗是获得性 TTP 抢救成功的关键。