中文摘要：

目的：探讨血栓性血小板减少性紫癜（TTP）的临床特点和治疗策略,提高TTP的诊疗水平。方法：回顾性分析57例TTP患者的临床表现、实验室检查、治疗方法、疗效和预后。结果：57例患者中,男16例,女41例,中位发病年龄38岁,49例表现为三联征,22例表现为五联征。血小板、血红蛋白及乳酸脱氢酶均值分别为（16.83±13.79）×109/L,（65.11±19.01）g/L和（1 169.69±668.27）U/L。外周血红细胞碎片比例为1.3%~31.0%。使用血浆置换＋糖皮质激素及血浆置换＋糖皮质激素＋免疫抑制剂的有效率分别为71.43%（25/35）和100%（13/13）;9例难治或复发的患者加用利妥昔单抗治疗,有效率为100%（9/9）。57例患者中,9例死亡,11例复发。死亡组与存活组在血红蛋白（P=0.027）、乳酸脱氢酶（P=0.024）、尿素氮（P=0.004）、年龄（P=0.009）、严重神经系统症状（P=0.037）、昏迷（P=0.023）和神经系统症状不断加重（P=0.000）方面差异有统计学意义,其中神经系统症状不断加重是TTP的独立预后因素（P=0.025）。复发组与非复发组年龄差异有统计学意义（P=0.005）。结论：TTP患者多数以三联征起病,血浆置换联合糖皮质激素是其标准治疗方案。对于难治复发的患者,利妥昔单抗可以提高有效率。神经系统症状不断加重是TTP的不良预后因素。

英文摘要：

Objective：To explore clinical characteristics and therapeutic strategies of the thrombotic thrombocytopenic purpura（TTP）patients.Method：Clinical manifestations,laboratory examinations,therapeutic methods,curative effects and prognosis of 57 TTP patients were retrospectively analyzed.Result：There were 16 male patients and 41 female patients.The medium onset age was 38 years old.Forty-nine patients had the Triad syndrome and 22 patients had the Quinary syndrome.The average counts of PLT,HB and LDH were（16.83±13.79）×109/L,（65.11±19.01）g/L,and（1 169.69±668.27）U/L,respectively.The percentage of erythrocyte fragments ranged from 1.3%to 31.0%.The effective rates of using glucocorticoids＋plasma exchange（PE）and glucocorticoids＋PE＋immunosuppressors were 71.43%（25/35）and 100%（13/13）,respectively.Nine refractory or relapsed patients used rituximab additionally and the effective rate was 100%（9/9）.Among the 57 patients,there were 9patients dead,11 patients relapsed.HB（P=0.027）,LDH（P=0.024）,BUN（P=0.004）,age（P=0.009）,severe neurological symptoms（P=0.037）,coma（P=0.023）and aggravated neurological symptom（P=0.000）had significant difference between the dead group and the live group.And aggravated neurological symptom（P=0.025）was the independent prognostic factor.Age（P=0.005）had significant difference between the relapsed group and the un-relapsed group.Conclusion：Most of the TTP patients occur with the Triad syndrome,and PE combining glucocorticoids is the standard therapeutic method.For the refractory and relapsed TTP patients,rituximab can improve the remission rate.Aggravated neurological symptom is a poor prognostic factor to TTP.