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中华细胞与干细胞杂志(电子版) ›› 2019, Vol. 09 ›› Issue (06) : 358 -362. doi: 10.3877/cma.j.issn.2095-1221.2019.06.007

所属专题: 文献

综述

异基因造血干细胞移植后并发自身免疫性溶血性贫血的治疗进展
龚悦1, 陈静1,()   
  1. 1. 200127 上海交通大学附属上海儿童医学中心
  • 收稿日期:2019-07-01 出版日期:2019-12-01
  • 通信作者: 陈静

Progress in the treatment of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation

Yue Gong1, Jing Chen1,()   

  1. 1. Department of Hematology, Shanghai Children's Medical Center, Shanghai Jiao Tong University, Shanghai 200127, China
  • Received:2019-07-01 Published:2019-12-01
  • Corresponding author: Jing Chen
  • About author:
    Corresponding author: Chen Jing, Email:
引用本文:

龚悦, 陈静. 异基因造血干细胞移植后并发自身免疫性溶血性贫血的治疗进展[J]. 中华细胞与干细胞杂志(电子版), 2019, 09(06): 358-362.

Yue Gong, Jing Chen. Progress in the treatment of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation[J]. Chinese Journal of Cell and Stem Cell(Electronic Edition), 2019, 09(06): 358-362.

异基因造血干细胞移植(HSCT)后自身免疫性溶血性贫血(AIHA)是HSCT后并不少见的并发症,其发病率约1﹪~ 6﹪,不同于一般的AIHA,HSCT后AIHA发病机制尚未完全明确,可能与HSCT后受者体内免疫失调相关。危险因素与移植患者年龄小、非恶性疾病、使用无关供者、半相合供者移植、脐血移植、去T细胞移植及移植后并发慢性移植物抗宿主病(GVHD)等有关。皮质激素作为一线治疗,疗效有限,难以持续缓解,需联合使用利妥昔单抗(RTX)、大剂量丙种球蛋白等,甚至需要联合霉酚酸酯、环磷酰胺、西罗莫司、阿伦单抗、依库丽单抗或蛋白酶体抑制剂硼替佐米等免疫抑制剂治疗,部分患者需行血浆置换,偶有行脾切除术者。移植后AIHA总死亡率常高达50﹪,总体预后差于单纯AIHA。该综述旨在总结HSCT后并发AIHA的最新治疗进展,供临床医师参考。

Autoimmune hemolytic anemia (AIHA) is not a rare complication of allogeneic hematopoietic stem cell transplantation (HSCT) , the incidence of AIHA after HSCT ranges between about 1﹪ to 6﹪. Different from common AIHA, the pathogenesis of AIHA after HSCT is not completely clear, which may be related to immune dysregulation after HSCT. Risk factors include young age, non-malignant disease, use of unrelated donors, haploidentical transplant, cord blood transplantation, T-cell depletion and presence of chronic graft-versus-host disease (GvHD) . As a first-line therapy, steroids often have limited therapeutic effects and are difficult to sustain remission. Therefore, they often need a combination of rituximab, large dose of immunoglobulins, even need to further joint immunosuppressive agents such as mycophenolate mofetil, cyclophosphamide, sirolimus, alemtuzumab, eculizumab, proteasome inhibitors bortezomib and so on. Some patients receive plasma exchange, occasionally splenectomy. The overall prognosis is significantly worse than primary AIHA, and the total mortality of AIHA after HSCT is often up to 50﹪. This review aims to summarize the latest treatment progress of AIHA after HSCT for clinicians' reference.

图1 异基因造血干细胞移植后自身免疫性溶血性贫血的治疗
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