论文部分内容阅读
目的探讨异基因造血干细胞移植(allo-HSCT)治疗儿童噬血细胞淋巴组织细胞增生症(HLH)的疗效与治疗经验。方法对8例HLH患儿行allo-HSCT,观察造血重建、移植相关并发症及疗效。结果移植后10～21d(中位时间14.3d)白细胞>1.0×109/L;移植后14～29d(中位时间19.5d)血小板>20×109/L。3例发生Ⅰ度急性移植物抗宿主病(GVHD),1例发生Ⅱ度急性GVHD,其中1例发生慢性GVHD。3例发生肺部感染,其中2例为巨细胞病毒感染,1例发生肠道感染,1例合并颜面部蜂窝组织炎;1例发生肝静脉闭塞病(VOD),1例出现出血性膀胱炎。随访6个月～5年,6例患儿无病生存,2例分别死于VOD和脓毒血症。结论 allo-HSCT是治疗HLH的可靠方法,在充分考虑移植风险和积极准备的基础上可实施造血干细胞移植。
Objective To investigate the curative effect and therapeutic experience of allo-HSCT in children with hemophagocytic lymphohistiocytosis (HLH). Methods Eight cases of HLH children underwent allo-HSCT, observed hematopoietic reconstitution, transplantation-related complications and efficacy. Results Leukocytes> 1.0 × 109 / L after 10 ~ 21 days (median time 14.3 days) and platelets> 20 × 109 / L after 14 ~ 29 days (median 19.5 days) after transplantation. Three patients developed grade A acute graft-versus-host disease (GVHD), one developed grade II acute GVHD, and one developed chronic GVHD. Three patients developed pulmonary infection, of which 2 were CMV infection, 1 had intestinal infection, 1 had facial cellulitis; 1 developed hepatic veno-occlusive disease (VOD) and 1 developed hemorrhagic cystitis . Six months to five years of follow-up, 6 children survived without disease and 2 died of VOD and sepsis respectively. Conclusion Allo-HSCT is a reliable method for the treatment of HLH. Hematopoietic stem cell transplantation can be performed on the basis of full consideration of the risk of transplantation and active preparation.