白细胞介素-6阻滞治疗在噬血细胞性淋巴组织细胞增生症中的研究新进展

Research advances on interleukin-6 blocking therapy in treatment of hemophagocytic lymphohistiocytic hyperplasia

噬血细胞性淋巴组织细胞增生症(HLH)亦称噬血细胞综合征(HPS),是由多种原发或继发因素导致淋巴细胞和组织细胞异常激活,并分泌大量炎性细胞因子,引起严重甚至危及生命的过度炎症反应综合征。白细胞介素(IL)-6是HLH发生细胞因子释放综合征(CRS)的核心细胞因子,通过影响穿孔素及颗粒酶B表达降低细胞毒性T淋巴细胞(CTL)和自然杀伤(NK)细胞活性,参与HLH的发生、发展。因此,阻滞IL-6及其信号通路成为治疗HLH的新方法。笔者通过对IL-6及其信号通路在HLH中的致病机制、IL-6阻滞治疗HLH患者及其相关不良反应的研究新进展进行阐述。

Hemophagocytic lymphohistiocytic hyperplasia(HLH),also known as hemophagocytic syndrome(HPS),is a syndrome of excessive inflammatory response caused by a variety of primary or secondary factors that lead to abnormal activation of lymphocytes and histiocytes and secretion of large amounts of inflammatory cytokines,resulting in a serious or even life-threatening condition.Interleukin(IL)-6 is a central cytokine in development of cytokine release syndrome(CRS)in HLH,and is involved in development and progression of HLH by affecting expression of perforin and granzyme B to reduce cytotoxic T lymphocyte(CTL)and natural killer(NK)cell activity.Therefore,blocking IL-6 and its signalling pathway has become a novel therapeutic method for HLH.This article focus on the pathogenic mechanisms of IL-6 and its signalling pathway in HLH,the progress of research on IL-6 blockade in treatment of patients with HLH and its associated adverse effects.