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目的 分析获得性血栓性血小板减少性紫癜(TTP)患者的临床特征、治疗策略及转归.方法 回顾性分析51例获得性TTP患者的临床资料.采用GraphPad Prism5软件对数据资料进行分析.结果 51例患者中男17例、女34例,中位年龄41(13～90)岁；仅18例(35.29％)表现为典型五联征,以血小板减少(100.00％)、微血管病性溶血性贫血(92.16％)、神经精神症状(88.24％)较为多见,而发热(72.55％)及肾脏损害(70.59％)相对少见.37例患者进行血浆血管性血友病因子裂解蛋白酶(ADAMTS 13)活性检查,其中31例(83.78％)ADAMTS13活性＜5％.36例(70.59％)患者治疗有效,血浆置换和血浆输注有效率为72.3％. 15例(29.41％)患者死亡,8例(22.22％)复发.死亡患者发病年龄、治疗前总胆红素水平均高于治疗有效患者[(50.1±18.9)岁对(37.5±14.5)岁,P=0.008;(63.7±39.7)μmol/L对(43.3±23.5)μmol/L,P=0.036],而体温、白细胞数、血红蛋白、血小板计数、血肌酐及LDH差异均无统计学意义(P＞0.05).结论 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TTP的临床诊断.血浆置换疗效较好,联合糖皮质激素、免疫抑制剂、利妥昔单抗可进一步降低病死率及复发率.高龄及总胆红素增高患者预后欠佳.

Objective To comprehensively analyze the clinical characteristics,treatment strategies and outcome of patients with thrombotic thrombocytopenic purpura (TTP).Methods A retrospective survey of 51 TTP patients confirmed in our database.Relevant statistical analyzes were performed by GraphPad Prism 5 software.Results 51 cases of patients with acquired TTP were identified as idiopathic TTP.In our study,only 18 cases (35.29％) had typical pentalogy of TTP,where thrombocytopenia (100.00％),microangiopathic hemolytic anemia (92.16％) and neurologic abnormalities (88.24％) were more common than fever (72.55％) and renal abnormalities (70.59％).Plasma ADAMTS13 activity was detected in 37 patients with TTP with ADAMTS 13 deficiency confirmed in 31 patients (83.78％).Plasma exchange with response of 72.3％ was still the preferred strategy in TTP with individuation.Among 36 survival TTP patients,8 patients (22.22％) relapsed.15 patients (29.41％) died in our study.The mean ages of resonders and deaths were of (37.5± 14.5) and (50.1 ± 18.9) respectively; whereas total bilirubin level of resonders and deaths were of (43.3 ±23.5) μmol/L and (63.7 ± 39.7) μmol/L respectively,the differences were statistically significant.Conversely,body temperature,WBC,HGB,PLT,serum creatinine and LDH showed no significant differences (P＞0.05).Conclusion The diagnosis of TTP was based on comprehensive analysis of clinical manifestations.Plasma ADAMTS 13 activity test had a higher clinical practical value.The therapeutic alliance with corticosteroids,immunosuppressive agents and Rituximab significantly improved its outcome.The age and high total bilirubin level at onset were associated with less sensitive to plasmapheresis and poor prognosis.