异基因造血干细胞移植后同时发生纯红细胞再生障碍性贫血和EVANS综合征1例并文献复习

Pure red cell aplasia and Evans syndrome after unrelated hematopoietic stem cell transplantation in a patient with severe aplastic anemia: a case report and literature review

目的:通过对异基因造血干细胞移植(HSCT)后同时发生纯红细胞再生障碍性贫血(PRCA)和EV-ANS综合征等造血功能紊乱发病机制的认识,深入了解HSCT过程中特殊的免疫功能状态,从而合理制定预处理方案和干预措施。方法:报道1例重症再生障碍性贫血(SAA)行非血缘HSCT后同时发生PRCA和EVANS综合征的临床及成功治疗经过并复习相关文献。结果:患者经减免疫抑制剂,应用利妥昔单抗,输注供者G-CSF动员的外周血干细胞(DSI)等治疗后,PRCA和EVANS综合征得到成功纠正,血常规恢复正常。结论:HSCT后同时发生PRCA和EVANS综合征十分罕见,目前世界上未见报道。其发生与非清髓预处理对不同淋巴细胞亚群的清除程度不同,HSCT后B和T,CD4+和CD8+等淋巴细胞亚群重建速度不一致等因素有关。了解以上特点,有利于制定更加合理的预处理方案,并处理相关并发症。

Objective：To study the etiology of pure red cell aplasia（PRCA） and Evans syndrome after allogeneic hematopoietic stem cell transplantation（allo-HSCT）.Method：The clinical manifestations,laboratory examination,treatment and outcome of a patient with severe aplastic anemia（SAA） who developed PRCA and Evans syndrome simultaneously after unrelated-HSCT were presented.In addition,the relevant literatures were reviewed.Result：PRCA and Evans syndrome were resolved completely with combined treatments of immunosuppressant reduction,anti-CD20 monoclonal antibody application and G-CSF mobilized peripheral stem cells infusion from original donor.Conclusion：Simultaneous occurrence of PRCA and Evans syndrome is rare in patients after allo-HSCT.To our knowledge,no similar case has been reported worldwidely.It is caused by immune dysfunction after allo-HSCT.Therefore it is very helpful from this case to understand more about the immune reconstitution after allo-HSCT,to recognize the clinical features that related to immune dysfunction,to choose appropriate therapy,and to design the conditioning regimen with minimized immune dysfunction post allo-HSCT in order to improve the survival and quality of life.