84例成人Evans综合征临床资料分析

目的 探讨成人Evans综合征的初始临床特征,各种治疗方法 的疗效及病程.方法 对84例成人Evans综合征患者(男20例,女64例)应用激素、丙种免疫球蛋白、免疫抑制剂多药联合治疗,中位随访17.5(0.03～140)个月,观察患者起病特征及临床疗效.结果 所有患者均接受激素加或不加静脉丙种免疫球蛋白初始治疗.47例患者单用激素治疗,其中38例达完全缓解(CR)和部分缓解(PR).中位随访12个月,92.1%的患者复发.28例对激素耐药或出现严重出血的患者随后给予免疫抑制剂治疗,89.3%的患者获得CR或PR.中位随访8个月,84%患者复发.结论 Evans 综合征难治且易复发,联合治疗可能是治疗Evans综合征的有效手段.

A retrospective analysis of 84 adult patients with Evans syndrome in a single center

Objective To evaluate the initial clinical characteristics, the response to treatment, and the outcome in adult patients with Evans syndrome. Methods The clinical data of 84 adult patients (20 males, 64 females) with Evans syndrome diagnosed at our center between 1984 and 2007 were retrospectively analyzed. Results The patients were followed up for a median duration of 17.5 (0.03-140) months. All the patients initially received intravenous steroids with or without intravenous immunoglobulin (IVIG). Forty-seven patients were treated with corticosteroids alone initially. Complete remission (CR) and partial remission (PR) were achieved in 38 of the patients, but 92. 1% of them relapsed during a median follow-up of 12 months. Twenty-eight patients who were resistant to corticosteroids therapy or with severe bleeding were subsequently administered immunosupressive agents. CR and PR were obtained in 89.3% of them. Within a median follow-up of 8 months, 84% of these patients relapsed. Conclusions Evans syndrome is a chronic and easy to recurrent disease, which is often refractory to conventional therapy. Treatment with combination agents might be a useful therapeutic approach to the patients.