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目的:分析湿疹血小板减少伴免疫缺陷综合征(WAS)患儿脐血移植后的疗效和影响因素,为供体选择和受体移植的时机提供经验。方法:5例WAS患儿接受非血缘脐血干细胞移植,分析移植前营养状态、移植时年龄、HLA相合度、移植后移植物抗宿主病(GVHD)及感染因素对移植效果的影响,随访时间截止到2016年1月。结果:5例WAS患儿成功植入。粒细胞植入时间分别为15d、12d、11d、14d、15d,血小板植入时间分别为39d、16d、16d、34d、36d,HLA高分辨检测显示供受体相合度分别为8/10、10/10、5/10、10/10、10/10;其中3例患儿移植前有巨细胞病毒血症,移植后血小板植入缓慢,1例中度营养不良的患儿移植后有皮肤及肠道重度GVHD和反复重症感染。结论:移植前患儿巨细胞病毒血症和移植前营养状态是影响血小板植入和预后的主要因素,HLA高分辨位点相合程度对粒细胞和血小板植入及GVHD没有显著影响。
OBJECTIVE: To analyze the efficacy and influencing factors of cord blood transplantation in children with eczema thrombocytopenic syndrome (WAS) and to provide experience for the timing of donor selection and recipient transplantation. Methods: Five children with WAS received non-blood-derived cord blood stem cell transplantation. The effects of pre-transplantation nutritional status, age at transplantation, HLA matching, graft-versus-host disease (GVHD) and infection factors on graft outcome were analyzed. As of January 2016. Results: Five children with WAS were successfully implanted. The time of granulocyte implantation was 15d, 12d, 11d, 14d and 15d respectively, and the platelet engraftment time was 39d, 16d, 16d, 34d and 36d, respectively. / 10,5 / 10,10 / 10,10 / 10; 3 cases of children with cytomegalovirus before transplantation, slow platelet transplanted after transplantation, 1 case of moderate malnutrition in children with skin and Intestinal severe GVHD and recurrent severe infections. CONCLUSION: Pre-transplant cytokines and pre-transplant nutritional status are the major factors that influence platelet engraftment and prognosis. The degree of HLA HLA-matched sites has no significant effect on granulocyte and platelet transplants and GVHD.