CAJ | 학술논문

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。
목적：총결획득성혈전성혈소판감소성자전（TTP）환자적림상표현、실험실검사、치료급전귀。방법선취2013년1월—2014년12월하북의과대학제이의원수치적소유획득성 TTP 환자16례위연구대상。회고성분석환자적림상표현、실험실검사자료、혈장혈관성혈우병인자렬해단백매（ADAMTS 13）활성급기억제물검사결과、치료방법급료효。결과16례환자중，5례（31.3％）유전형적“오련정”。소유환자유불동정도적미혈관병성용혈성빈혈화혈소판계수（PLT）감소。14례환자행외주혈도편검사，10례가견파쇄홍세포，6례파쇄홍세포＞1％。15례환자유산탈경매（LDH）승고，소유환자간접담홍소（DBIL）승고。16례환자 ADAMTS 13활성균감저，기중15례ADAMTS 13활성＜10％；ADAMTS 13억제물검측결과균위양성。16례환자중，2례우치료전사망，1례방기치료，기여13례급여혈장치환（PE）연합당피질격소치료，기중2례우치료개시24 h 내사망，11례환자치료유효。결론획득성 TTP 진단주요의고림상특정，포괄미혈관병성용혈성빈혈、PLT 감소、신경계통이상、신공능이상、발열， ADAMTS 13활성중도감저급기억제물양성시진단획득성 TTP 적중요실험실의거，진조개시 PE 치료，동시연합면역억제제치료시획득성 TTP 창구성공적관건。
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.