获得性血栓性血小板减少性紫癜患者的临床特征分析

Clinical Characteristics of Acquired Thrombotic Thrombocytopenic Purpura

背景获得性血栓性血小板减少性紫癜(TTP)发病机制尚不明确,病死率极高,因此了解获得性TTP的临床特征有助于帮助医务工作者早诊断、早治疗,降低其病死率。目的分析获得性TTP患者的临床特征、治疗策略及转归。方法回顾性分析2007年1月-2017年7月在苏州大学附属第一医院住院的符合纳入标准的82例获得性TTP患者的临床资料,包括发病诱因、临床表现、实验室指标、人血管性血友病因子裂解蛋白酶(ADAMTS 13)活性和抑制物测定情况、治疗及转归。结果患者发病的诱因分类中,以无明显诱因多见(68.29%),其次依次为感染(10.98%),其中2例系统性红斑狼疮、1例红皮病、1例银屑病、1例舍格伦综合征)、自身免疫性疾病(6.10%)、分娩诱发(6.10%)。临床表现:血小板减少症中以皮肤瘀点瘀斑(58.54%)、血尿(29.27%)为主,微血管病变性溶血性贫血中以贫血貌(100.00%)、皮肤及巩膜黄染(43.90%)为主,神经精神症状中以昏迷((34.15%)、抽搐(26.83%)为主,肾功能损害中以蛋白尿(68.29%)为主,发热占54.88%;出现"五联征"的有19例(23.17%)。82例患者均进行ADAMTS 13活性检测,其中65例患者ADAMTS 13活性<10%,抑制物阳性者38例。82例TTP患者中54例治疗好转后出院(有效组),28例患者死亡(死亡组)。死亡组患者体温、丙氨酸氨基转移酶、天冬氨酸氨基转移酶、乳酸脱氢酶、血清总胆红素、血清肌酐、血清尿素氮高于有效组,开始行血浆置换(PE)的时间长于有效组,PE次数少于有效组(P<0.05)。结论获得性TTP患者的诊断依赖临床资料综合分析。血浆ADAMTS 13活性检测有助于获得性TTP的临床诊断。PE疗效较好,联合糖皮质激素和免疫抑制剂可进一步降低病死率及复发率。

Background The pathogenesis of acquired thrombotic thrombocytopenic purpura(TTP)is not clear and the mortality rate is very high.Therefore,understanding the clinical characteristics of TTP can help medical workers to diagnose and treat TTP early and reduce its mortality.Objective To comprehensively analyze the clinical characteristics,treatment strategies and outcome of patients with acquired TTP.Methods We enrolled 82 inpatients with acquired TTP from the First Affiliated Hospital of Soochow University from January 2007 to July 2017 in accordance with the inclusion criteria,and retrospectively analyzed their clinical data,including precipitating factors,clinical manifestations,laboratory indicators,ADAMTS 13 activity and inhibitor titre,treatment and outcome.Results Unapparent causes accounted for the largest percentage of the total precipitating factors(68.29%),followed by infections(10.98%,including 2 cases of systematic lupus erythematosis,1 case of erythroderma,1 case of psoriasis,and 1 case of Sjogren's syndrome),autoimmune disease(6.10%),and childbirth(6.10%).Clinical manifestations:thrombocytopenia was mainly manifested as skin ecchymosis(58.54%),and hematuria(29.27%);microangiopathic hemolytic anemia was mainly manifested as appearance of anemia(100.00%),yellowing appearance of skin and mucous membranes and sclera(43.90%);neuropsychiatric symptoms mainly included coma(34.15%)and convulsions(26.83%);renal dysfunction was manifested mostly as proteinuria(68.29%)and fever(54.88%);typical pentalogy of TTP was found in 23.17%(19/82)of the total cases.Plasma ADAMTS 13 activity and inhibitor titre:ADAMTS 13 activity was detected in all cases,and 65 were found with activity<10%,38 with positive ADAMTS 13 inhibitor.Treatment and outcome:54 cases were improved after treatment,and were discharged,other 28 died.The died had higher mean temperature,ALT,AST,LDH,serum total bilirubin,creatinine,and urea,shorter time of PE,and less number of PEs than the survived(P<0.05).Conclusion The diagnosis of acquired TTP was based on comprehensive analysis of clinical data.Plasma ADAMTS 13 activity assay contributes to the clinical diagnosis.Plasma exchange has good efficacy,and its combined use with corticosteroids and immunosuppressive agents may further reduce the mortality and likelihood of recurrence of this disease.