目的 探究血栓性血小板减少性紫癜(TTP)患者的临床特征及诊治方法.方法 对2010年1月至2017年12月收住本院诊治的30例TTP患者的一般情况、临床表现、实验室检查、诊治方法及转归等进行回顾性研究.结果 18例(60.00%)患者表现为典型TTP"五联征".最终死亡10例(33.33%),复发1例(3.33%),其中26例(86.67%)患者行血浆置换(PE),12例(40%)患者完善血管性血友病因子裂解蛋白酶(ADAMTS13)活性检测并提示活性<5%.治疗有效组与死亡组患者间接胆红素水平存在显著差异(P=0.008).结论 TTP患者症状复杂多变,血浆置换仍是治疗的首选措施,血管性血友病因子裂解蛋白酶(ADAMTS13)活性检测有助于TTP的诊断,间接胆红素升高水平可能与预后相关.%Objective To analyze the clinical characteristics,treatment strategies and outcome of the patients with thrombotic thrombocytopenic purpura(TTP). Methods Clinical features,laboratory tests,treatment protocols and outcome of 30 cases with TTP were analyzed retrospectively and observed in our hospital from January 2010 to December 2017. Results In our study,18 cases(60%)had typical pentalogy of TTP. 10 patients (33.33%)died,and one of the remaining 20 cases relapsed.26 patients(86.67%)underwent plasma exchange(PE).12 patients(40%)were tested for plasma ADAMTS13 and all of them confirmed ADAMTS13 deficiency. There were statistically significant differences in the level of indirect bilirubin between the death group and the effective group(P=0.008). Conclusion The symptoms of TTP patients are changeable and complicated. Plasma exchange is considered to be the preferred treatment for TTP. ADAMTS13 activity test has a higher clinical practical value. The level of indirect bilirubin may be associated with poor prognosis.
展开▼
