摘要
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 analyse the relapse rate and risk factors of autoimmune hemolytic anemia(AIHA) and Evans syndrome. Methods Fifty two cases of AIHA and Evans syndrome in remission being followed up for 1～14 years (median time 3.8 years) were analysed for relapse rate. The risk factors of relapse were analysed by case-control study. Results The total relapse rate of these AIHA and Evans syndrome patients was 57.7%, and the median remission duration to the first relapse was 9 months.The relapse rates in patients with negative Coombs test ,warm autoantibodies and both of warm and cold autoantibodies were 30.8%(4/13), 54.0%(13/24) and 86.7% (13/15), respectively. The relapse rate in patients with cold antibody was the highest(P<0.05).The relapse rate in patients with antibody titer≥100 was 92.9%(13/14) and was higher than that in patients with antibody titer<100 [59.5%(13/22)] (P<0.05). Patients treated with prednisone and cyclosporin relapsed less than those treated with prednisone alone, and the relapse was related to the therapy course of prednisone and CsA. Conclusion Because of the high relapse rate, AIHA and Evans syndrome should be treated according to the class of autoantibodies, and with longer course of prednisone and cyclosporin and prophylaxis of infection.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2003年第10期534-537,共4页
Chinese Journal of Hematology
