CAJ | 학술논문

目的：分析含高剂量 CD34＋细胞的非血缘脐血移植治疗急性粒细胞白血病合并骨髓增生综合征（AML/MDS）后发生原发植入失败的原因。方法1例4岁女孩在四川大学华西第二医院儿科血液肿瘤科被诊断为 AML/MDS。患儿经诱导及巩固治疗获完全缓解后，行无血缘人类白细胞抗原（HLA）部分不相合脐血移植。观察患儿术后造血重建及移植相关并发症情况。结果患儿术后发生原发植入失败，再次进行血缘间的半相合造血干细胞移植，期间患多药耐药鲍曼不动杆菌败血症，于第2次移植后7 d 死于呼吸衰竭。结论含高剂量 CD34＋细胞脐血造血干细胞并不能抵消 HLA配型不合的缺陷。患儿原发植入失败可能与患儿移植前存在长期血小板输注无效及潜在免疫异常，尤其是移植前产生抗-HLA 供者特异性抗体有关。
목적：분석함고제량 CD34＋세포적비혈연제혈이식치료급성립세포백혈병합병골수증생종합정（AML/MDS）후발생원발식입실패적원인。방법1례4세녀해재사천대학화서제이의원인과혈액종류과피진단위 AML/MDS。환인경유도급공고치료획완전완해후，행무혈연인류백세포항원（HLA）부분불상합제혈이식。관찰환인술후조혈중건급이식상관병발증정황。결과환인술후발생원발식입실패，재차진행혈연간적반상합조혈간세포이식，기간환다약내약포만불동간균패혈증，우제2차이식후7 d 사우호흡쇠갈。결론함고제량 CD34＋세포제혈조혈간세포병불능저소 HLA배형불합적결함。환인원발식입실패가능여환인이식전존재장기혈소판수주무효급잠재면역이상，우기시이식전산생항-HLA 공자특이성항체유관。
Objective To analysis the cause of primary graft failure of unrelated cord blood transplantation with high-dose of CD34 + cells in treatment of acute myelocytic leukemia (AML) /myelodysplastic syndrome (MDS).Methods A 4-year-old girl was diagnosed AML/MDS at the Department of Pediatric Hematology and Oncology of West China Second University Hospital of Sichuan University. She presented completely remission after induction and consolidation chemotherapy.She received unrelated partially human leukocyte antigen (HLA)-mismatched cord blood transplantation.We investigated the treatment outcomes of UCBT and associated complications.Results The patient suffered primary graft failure and then received secondary haploidentical hematopoietic stem cell transplantation (HSCT)from her mother.However,she suffered fatal multiresistant Acinetobacter spp septicemia.She died due to respiratory failure on 7 d after the second transplantation.Conclusions In this case,hematopoietic stem cells with high dose of CD34 + cells could not overcome the risk of primary graft failure and HLA disparity.The patient's primary graft failure was associated with platelet transfusion refractoriness and potent immunologic dysfunction,especially the anti-HLA donor specific antibodies before unrelated cord blood transplantation.