Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory clinical syndrome of uncontrolled immune response which results in hypercytokinemia due to underlying primary or secondary immune defect. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only cure therapy for primary HLH and recurrent/refractory hemophagocytic lymphohistiocytosis. Compared with children HLH, adult HLH is a much more heterogeneous syndrome requiring a more individualized protocol depending on the underlying trigger, disease severity and genetic background. At present, there remain controversies in various aspects including indications of haematopoietic cell transplantation (HCT), conditioning regimen, efficacy and prognosis. This article will review the recent advances of allo-HSCT in the treatment of adult HLH based on the above issues.
题目: 异基因造血干细胞移植治疗成人噬血细胞综合征的研究进展.
摘要: 噬血细胞综合征（HLH）是一种由各种诱因致免疫细胞过度活化，分泌大量炎症因子的危重疾病，分为原发性和继发性。异基因造血干细胞移植（allo-HSCT）是治疗原发性HLH及复发难治性HLH的唯一治愈手段，相比于儿童HLH，成人HLH更具有异质性，需根据潜在诱因、疾病严重程度及遗传背景等制定个体化的移植方案。目前，造血细胞移植治疗成人HLH的适应证、预处理方案、疗效及预后等问题尚未完全达成共识。本文将结合上述问题对allo-HSCT治疗成人HLH的最新研究进展作一综述.
Keywords: adult; allogeneic hematopoietic stem cell transplantation; hemophagocytic lymphohistiocytosis; treatment.