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目的探讨血浆置换(PE)治疗获得性血栓性血小板减少性紫癜(TTP)的疗效及影响因素。方法回顾性分析12例TTP患者的临床特征及实验室资料、PE的实施、疗效及影响因素。结果12例TTP中特发性8例,继发性4例,均有微血管性溶血性贫血(MHA)表现、血小板显著减少、明显的神经征象、肾脏损害和发热。给予PE、血浆输注(PI)及联合免疫抑制剂治疗。11例PI2000(400~5400)ml,PE2~10次(中位数2次);12例PE血浆4900(2900~25000)ml;11例PI+PE血浆总量3300~27000ml。2例>20000ml者5(3~22)d起效。生存率83.3%,特发性、继发性各1例死亡(1例与PE相关)。结论重症TTP者应及早行PE或PI+PE及联合免疫抑制治疗,以提高缓解率和治愈率,降低复发率。
Objective To investigate the efficacy and influencing factors of plasma exchange (PE) in the treatment of acquired thrombotic thrombocytopenic purpura (TTP). Methods Retrospective analysis of 12 cases of TTP patients clinical features and laboratory data, the implementation of PE, efficacy and influencing factors. Results TTP 12 cases of idiopathic 8 cases, secondary 4 cases, all have microvascular hemolytic anemia (MHA) performance, significant reduction of platelets, significant neurological signs, kidney damage and fever. Given PE, plasma infusion (PI) and combined immunosuppressive therapy. 11 cases of PI2000 (400 ~ 5400) ml, PE2 ~ 10 times (median 2 times); 12 cases of PE plasma 4900 (2900 ~ 25000) ml; 11 cases of PI + PE plasma total 3300 ~ 27000ml. 2 cases> 20000ml 5 (3 ~ 22) d onset. Survival rate of 83.3%, idiopathic, secondary to each one death (1 case of PE-related). Conclusion Severe TTP should be treated with PE or PI + PE and combined immunosuppressive therapy as early as possible to improve the remission rate and cure rate and reduce the recurrence rate.