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[复发性/难治性自身免疫性溶血性贫血脾切除术的疗效]

[Outcomes of splenectomy in relapsed/refractory autoimmune hemolytic anemia].

作者信息

Fan S B, Wang Z J, Mao Q, Tong C F, Zhai W T, Zheng Y Z, Sun C X, Shi J

机构信息

Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2019 Feb 14;40(2):132-136. doi: 10.3760/cma.j.issn.0253-2727.2019.02.007.

Abstract

To evaluate the outcomes of splenectomy in the treatment of relapsed/refractory autoimmune hemolytic anemia (AIHA). 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. 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. 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.

摘要

评估脾切除术治疗复发/难治性自身免疫性溶血性贫血(AIHA)的疗效。对我院30例接受脾切除术治疗的复发/难治性AIHA患者进行回顾性分析。随访术前和术后血常规指标及反应情况。30例复发/难治性AIHA患者中,20例为单纯AIHA(包括13例温抗体型AIHA、2例温冷双抗体型AIHA和5例Coombs阴性AIHA),10例为Evans综合征。术后10 - 14天评估短期反应,20例可评估治疗效果的病例中短期反应的总缓解率(ORR)为90%[完全缓解(CR)12例,部分缓解(PR)6例]。在13例有长期随访数据的患者中,除3例Evans综合征患者死亡(2例对脾切除术无效,1例术后复发)外,10例复发/难治性单纯AIHA患者在6个月和12个月时的ORR分别为90%(9/10)和70%(7/10),中位随访时间为14(4 - 156)个月。随访结束时,3例患者维持CR超过3年。脾切除术作为复发/难治性AIHA二线治疗的短期反应令人满意,脾切除术1年的长期缓解率可达70%。约三分之一的患者可维持持续缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/541c/7342661/9af7962f0404/cjh-40-02-132-g001.jpg

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