Successful treatment of severe refractory autoimmune hemolytic anemia after hematopoietic stem cell transplant with abatacept
Successful treatment of severe refractory autoimmune hemolytic anemia after hematopoietic stem cell transplant with abatacept
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DOI：
10.1111/trf.14907
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发表时间：
2018-09-01
期刊：
影响因子：
2.9
通讯作者：
Ngwube, Alexander
中科院分区：
文献类型：
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作者：
Hess, Jennifer;Su, Leon;Ngwube, Alexander
背景造血干细胞移植（HSCT）后，自身免疫性溶血性贫血（AIHA）是一种已知且相当常见的并发症。常规治疗包括皮质类固醇、静脉注射免疫球蛋白、脾切除术和最近描述的单克隆抗体的使用通常是难治的。高发病率与这些严重的持续性病例阐明了替代疗法可用于treatment.Study设计和METHODS的差距，我们描述了成功使用阿巴西普严重难治性AIHA HSCT后在3 patients. CRITTSThree小儿难治性AIHA异基因干细胞移植后患者被观察到对多种免疫抑制疗法无反应，导致持续输血依赖。阿巴西普，一种融合蛋白，抑制T细胞活化结合CD 80/CD 86抗原呈递细胞（APC），从而阻断所需的CD 28 APC和T细胞之间的相互作用，治疗导致在决议的溶血.CONCLUSIONAbatacept可能提供显着的临床效益，在管理AIHA HSCT后。
BACKGROUNDAfter hematopoietic stem cell transplantation (HSCT) autoimmune hemolytic anemia (AIHA) is a known and fairly common complication. It is often refractory to conventional therapies including corticosteroids, intravenous immunoglobulin, splenectomy, and the more recently described use of monoclonal antibodies. The high morbidity associated with these severe persistent cases elucidates the gaps in alternative therapies available for treatment.STUDY DESIGN AND METHODSWe described the successful use of abatacept for severe refractory AIHA after HSCT in three patients.RESULTSThree pediatric patients with refractory AIHA after allogeneic stem cell transplantation were observed to be unresponsive to multitude immunosuppressive therapies, resulting in persistent transfusion dependency. Treatment with abatacept, a fusion protein that inhibits T-cell activation by binding to CD80/CD86 on antigen-presenting cells (APCs), thus blocking the required CD28 interaction between APCs and T cells, resulted in the resolution of hemolysis.CONCLUSIONAbatacept may provide significant clinical benefit in the management of AIHA after HSCT.