CAJ | 학술논문

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.
목적 분석획득성혈전성혈소판감소성자전(TTP)환자적림상특정、치료책략급전귀.방법 회고성분석51례획득성TTP환자적림상자료.채용GraphPad Prism5연건대수거자료진행분석.결과 51례환자중남17례、녀34례,중위년령41(13～90)세；부18례(35.29％)표현위전형오련정,이혈소판감소(100.00％)、미혈관병성용혈성빈혈(92.16％)、신경정신증상(88.24％)교위다견,이발열(72.55％)급신장손해(70.59％)상대소견.37례환자진행혈장혈관성혈우병인자렬해단백매(ADAMTS 13)활성검사,기중31례(83.78％)ADAMTS13활성＜5％.36례(70.59％)환자치료유효,혈장치환화혈장수주유효솔위72.3％. 15례(29.41％)환자사망,8례(22.22％)복발.사망환자발병년령、치료전총담홍소수평균고우치료유효환자[(50.1±18.9)세대(37.5±14.5)세,P=0.008;(63.7±39.7)μmol/L대(43.3±23.5)μmol/L,P=0.036],이체온、백세포수、혈홍단백、혈소판계수、혈기항급LDH차이균무통계학의의(P＞0.05).결론 TTP환자적진단의뢰림상자료종합분석.혈장ADAMTS 13활성검측유조우TTP적림상진단.혈장치환료효교호,연합당피질격소、면역억제제、리타석단항가진일보강저병사솔급복발솔.고령급총담홍소증고환자예후흠가.
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.