摘要
噬血细胞综合征(HPS)又被称为噬血细胞性淋巴组织细胞增多症(HLH),是多种因素引起的免疫介导的高炎症因子综合征。在HLH发病机制中γ干扰素发挥关键作用。依马利尤单抗(emapalumab)作为一种针对γ干扰素的人源化单克隆抗体,是首个被美国食品药品监督管理局(FDA)批准用于儿童和成年人原发性HLH的细胞因子靶向药物。近年,相关研究结果显示,依马利尤单抗治疗原发性和继发性HLH患者的疗效良好。笔者拟就依马利尤单抗治疗HLH的机制、临床疗效及安全性的研究现状进行阐述。
Hemophagocytic syndrome(HPS),also known as hemophagocytic lymphohistiocytosis(HLH),is an immune-mediated hyperinflammatory syndrome caused by multiple factors.Interferon-γplays a key role in pathogenesis of HLH.Emapalumab,a humanized anti-interferon-γmonoclonal antibody,is the first cytokine targeting drug approved by Food and Drug Administration(FDA)of United States for primary HLH in children and adults.In recent years,related studies on treatment of primary and secondary HLH patients with emapalumab achieved good therapeutic effect.This article elaborates on research status on mechanism,clinical efficacy and safety of emapalumab in treatment of HLH.
作者
孟广强
任婧
Meng Guangqiang;Ren Jing(Department of Hematology,First Affiliated Hospital of Shandong First Medical University&Shandong Provincial Qianfoshan Hospital,Jinan 250014,Shandong Province,China)
出处
《国际输血及血液学杂志》
2025年第2期101-106,共6页
International Journal of Blood Transfusion and Hematology
基金
山东省自然科学基金项目(ZR2024QH282)。
