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目的 了解自身免疫性溶血性贫血 (AIHA)和Evans综合征复发率及其相关因素。方法 对治疗后完全缓解的 5 2例AIHA和Evans综合征患者随访 1～ 14年 ,了解其复发率 ,并采用同期病例对照法 ,比较不同因素与复发的相关性。结果 总复发率为 5 7.7% ;首次复发中位时间为缓解后 9个月 ;Coombs试验阴性型复发率为 30 .8% (13例中 4例复发 ) ,温抗体型复发率为 5 4 .0 % (2 4例中 13例复发 ) ,冷温双抗体及冷抗体型复发率为 86 .7% (15例中 13例复发 ) ,合并冷抗体患者复发率明显高于其他两型 (P <0 .0 5 ) ;抗体效价和≥ 10 0的患者复发率为 92 .9% (14例中 13例复发 ) ,抗体效价和 <10 0者复发率为 5 9.1% (2 2例中 13例复发 ) ,随着抗体效价升高 ,复发率明显增高 (P <0 .0 5 ) ;反复感染者易复发 ;加入环孢菌素A(CsA)的治疗方案较传统单用激素方案复发率明显降低 (P <0 .0 1) ;复发与激素、CsA的疗程密切相关 (P <0 .0 1)。结论 AIHA和Evans综合征复发率较高 ;分型施治、减少感染、加用CsA、延长疗程可减少复发。
Objective To investigate the relapse rate of autoimmune hemolytic anemia (AIHA) and Evans syndrome and its related factors. Methods Fifty-two patients with AIHA and Evans syndrome who were completely relieved after treatment were followed up for 1 to 14 years to understand the relapse rate. The correlation of different factors with recurrence was compared with the same case-control method. The overall recurrence rate was 5 7.7%. The median time to initial relapse was 9 months after remission. The negative recurrence rate was 30.8% in Coombs test (4 cases relapsed in 13 cases), and the recurrence rate was 54%. 0% (13 of 24 cases relapsed). The relapse rate of cold and cold antibody was 86.7% (13 of 15 patients relapsed), and the relapse rate of patients with cold antibody was significantly higher than that of the other two types (P < 0.05). The antibody titers and recurrence rates of patients ≥10 were 92.9% (13 of 14 patients relapsed), and the antibody titers and the recurrence rate of <10 0 were 9.11% (2 of 22 13 cases relapsed). With the increase of antibody titer, the relapse rate was significantly higher (P <0.05); patients with recurrent infection were more likely to relapse; the treatment with cyclosporine A (CsA) than the conventional single hormone regimen Recurrence rate was significantly lower (P <0.01); relapse and hormone, CsA course of treatment are closely related (P <0.01). Conclusion The relapse rate of AIHA and Evans syndrome is high. The treatment of type and type of infection can reduce the infection, add CsA and extend the course of treatment to reduce the relapse.