减停免疫抑制剂诱发移植物抗宿主病治疗儿童ABO血型不合移植后纯红细胞再生障碍性贫血1例及文献复习  

Successful treatment of pure red cell aplasia with the reduction of immunosuppression to enhance GVHD in a patient after ABO-incompatible allogeneic haematopoietic stem cell transplantation:a case report and literature review

作　　者：翁文骏[1] 陈志峰[2] 夏修贤[2] 方建培[1] 

机构地区：[1]中山大学孙逸仙纪念医院,广州510120 [2]香港大学玛丽医院

出　　处：《中国小儿血液与肿瘤杂志》2012年第5期211-214,共4页Journal of China Pediatric Blood and Cancer

基　　金：广东省自然科学基金资助项目(No.9151051501000021)

摘　　要：目的探讨ABO血型不合移植后合并纯红细胞再生障碍性贫血(PRCA)的发生机制及治疗对策。方法报道1例重型地中海贫血患儿接受ABO血型不合的无关供者外周血造血干细胞移植(URD-PBSCT)后合并PRCA病例并进行文献复习。结果患儿接受ABO血型不合的URD-PBSCT获得成功后出现PRCA,依赖红细胞输注伴ABO血型抗体滴度显著升高,通过减停免疫抑制剂诱发移植物抗宿主病(GVHD)后,ABO血型抗体滴度显著下降,网织红细胞、血红蛋白和骨髓象随之恢复正常。结论免疫紊乱及ABO血型抗体是ABO血型不合移植后PRCA的最可能原因,减停免疫抑制剂诱发GVHD是治疗ABO血型不合移植后PRCA的一种优先考虑的选择,但需要注意GVHD的有效控制。（ PRCA ） after Objective To stu ABO-incompatible dy the pathogenesis and treatment strategy of pure red cell aplasia allogeneic hematopoietic stem cell transplantation （HSCT）. Methods A patient diagnosed as Beta thalassemia major was developed PRCA after HLA-identical but ABO-incompatible allogeneic HSCT. Results After transplantation, the patient developed PRCA dependent on the red blood cell infusion accompanied by the obvious high titer of ABO blood-group anti- body. PRCA was successfully treated with the reduction of immunosuppression to enhance GVHD and graft versus plasma cell effect. Consequently, the ABO blood-group antibody titer decreased obviously, and the reticulocyte, hemoglobin and bone marrow turned to normal as well. Conclusions Immune disorder and ABO blood-group antibody are the most ABO-incompatible allogeneic HSCT. The reduction important reasons of PRCA after HLA-identical but of immunosuppression to enhance GVHD and graft versus plasma cell effect is the priority selection for PRCA after ABO-incompatible allogeneic HSCT. A good control of GVHD should be monitored during the treatment.

关 键 词：ABO血型不合异基因造血干细胞移植 贫血 纯红细胞再生障碍性 移植物抗宿主病 

分 类 号：R725.5[医药卫生—儿科]