Objective: To evaluate the outcomes of splenectomy in the treatment of relapsed/refractory autoimmune hemolytic anemia (AIHA). Methods: Retrospective analysis was performed in 30 cases with relapsed/refractory AIHA who were treated with splenectomy in our hospital. The pre- and post-operative blood routine indexes and responses were followed up. Results: Among the 30 relapsed/refractory AIHA patients, 20 were pure AIHA (including 13 patients with warm antibody AIHA, 2 with warm-cold double antibody AIHA and 5 with Coombs negative AIHA) and 10 were Evans syndrome. The short-term response was evaluated 10-14 days after operation, and the overall response rate (ORR) of short-term response was 90% [12 cases in complete response (CR), 6 cases in partial response (PR)] in 20 therapeutic evaluable cases. Among 13 patients with long-term follow-up data, except 3 patients with Evans syndrome died (2 cases were refractory to splenectomy, 1 case relapsed after surgery), the ORR of 10 patients with relapsed/refractory pure AIHA at 6 months and 12 months were 90% (9/10) and 70% (7/10), respectively, with a median follow-up of 14 (4-156) months. At the end of follow-up, 3 cases had maintained CR for more than 3 years. Conclusion: The short-term response of splenectomy as a second-line treatment for relapsed/refractory AIHA is satisfactory, and long-term outcome of splenectomy is up to 70% at 1 year. Approximately one-third of patients could maintain sustained remission.
目的： 评估脾切术治疗复发/难治自身免疫性溶血性贫血（AIHA）患者的疗效。 方法： 回顾性分析中国医学科学院血液病医院30例行脾切除术治疗的复发/难治自身免疫性溶血性贫血（AIHA）患者的临床资料，评估手术疗效。 结果： 30例复发/难治AIHA患者中，单纯AIHA 20例（温抗体型13例，温冷双抗体型2例，Coombs阴性AIHA 5例），Evans综合征10例。脾切除术后10~14 d，20例患者可评价疗效，总有效率（ORR）为90%（完全缓解12例，部分缓解6例）。13例患者有长期随访资料，中位随访14（4~156）个月，截至随访终点，除3例Evans综合征患者死亡外（2例无效，1例复发），10例复发/难治AIHA患者术后6个月9例（90%）有效，术后12个月7例（70%）有效，其中3例患者已持续完全缓解3年以上。 结论： 脾切除术作为二线方案治疗复发/难治AIHA近期疗效好，1年远期ORR达到70%，约三分之一患者可维持长期持续缓解。.
Keywords: Autoimmune hemolytic anemia; Outcome; Refractory; Relapsed; Splenectomy.