异基因造血干细胞移植治疗噬血细胞综合征

噬血细胞综合征又称噬血细胞性淋巴组织细胞增生症,分为原发性和继发性两大类.对于家族性噬血细胞综合征(familial hemophagocytic lymphohistiocytosis,FHL)和难治性EB病毒相关噬血细胞综合征(EBV-HLH),异基因造血干细胞移植是目前唯一有效的治疗手段,但其鉴别诊断尤为困难,移植后多种并发症以及高病死率也受到越来越多人的关注.该文总结了近年来异基因造血干细胞移植治疗FHL和难治性EBV-HLH在诊断、预处理方案、移植后并发症、死亡原因分析及预后等方面的研究进展.

Allogeneic hematopoietic stem cell transplantation for hemophagocytic syndrome

Hemophagocytic syndrome,also called hemophagocytic lymphohistiocytosis,includes primary hemophagocytic lymphohistiocytosis and secondary hemophagocytic lymphohistiocytosis.Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only effective therapy for familial hemophagocytic lymphohistiocytosis (FHL) and refractory EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH),but the differential diagnosis is still difficult.Meanwhile,much more complications and high mortality have attracted attention of many researchers.In this review,the recent advance in allo-HSCT for FHL and EBV-HLH about diagnosis,conditioning regimen,complication after HSCT,death causes and prognosis has been described.