噬血细胞综合征发病机制及治疗研究进展

Research progress of pathogenesis and treatment of hemophagocytic lymphohistiocytosis

噬血细胞综合征又称噬血细胞性淋巴组织细胞增生症(HLH),是一种进展迅速、危及生命的血液系统疾病。该病以炎性反应过度激活为特征,主要表现为组织器官内大量CD8+T淋巴细胞和巨噬细胞过度活化,进而产生大量细胞因子,如干扰素-γ、肿瘤坏死因子-α、白细胞介素-6、白细胞介素-18等,最终出现细胞因子风暴,造成组织损伤。HLH虽是一种良性组织细胞增生性疾病,但疾病进展快,临床表现多样,病死率高。近年来对HLH的发病机制研究较前深入,治疗方面也有了明显进展。现就HLH的最新机制及治疗进展作一综述。

Hemophagocytic syndrome, which also is called hemophagocytic lymphohistiocytosis(HLH), is a rapidly progressive and life-threatening hematological system diseases.The typical features of the disease is the exce-ssive activation of the inflammatory response, mainly performance that a lot of excessive activation CD8+ T cells and macrophages filtrated in tissues and organs, which can produce large amounts of cytokines, such as interferon-γ, tumor necrosis factor-α, interleukin-6 and interleukin-18, and which eventually led to the inflammatory cytokines storm and tissue damage.Although, HLH is a benign histiocytic disease, it can progress quickly, its clinical manifestations are diverse, with high mortality rate.In recent years, the research of HLH pathogenesis and treatment has been well-researched before.The latest pathogenesis and treatment are summarized in this article.