摘要
目的探讨异基因造血干细胞移植治疗噬血细胞综合征(HPS)的疗效。方法采用降低预处理强度的方案对2例HPS患者联合化疗后进行外周血干细胞移植。结果 2例患者白细胞、血小板如期植活。例1随访16个月,为完全供者造血,未出现移植物抗宿主病,合并有轻微白质脑病;例2移植前即有中枢受累,移植后中枢神经系统(CNS)复发失访。结论采用降低预处理强度的方案对无CNS受累的HPS患者进行外周血干细胞移植,疗效较好,对移植前已存在CNS可能采用清髓性预处理方案更好。
Objective To explore the effect of allogeneic hematopoietic stem cell transplantation （HSCT） for hemoph- agocytic syndrome（HPS）. Methods 2 patients with HPS who had received combined chemotherapy underwent peripheral blood HSCT after reduced-intensity conditioning. Results The 2 patients were all engrafted. 1 patient was alive for 16 months without evidence of disease and GVHD. The other one who had central nervous system （CNS） involved relapsed in CNS. Conclusion This study provides further evidence for proloned DFS after FLU/BU/ATG Allo-HSCT for HPS. The reduced-intensity condition- ing regimen is effective for HPS who had no CNS complications. Those who had CNS complications before HSCT, the myeloabla- tive regimen may be better.
出处
《实用癌症杂志》
2013年第4期353-356,共4页
The Practical Journal of Cancer
基金
国家自然科学基金项目(31200686)
关键词
噬血细胞综合征
造血干细胞移植
Allogene
Hematopoietic stem cell transplantation （HSCT）
Hemophagocytic syndrome （HPS）
