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γ干扰素及其抗体拮抗剂在噬血细胞性淋巴组织细胞增生症中的研究进展 被引量:2

The role of interferon-γ and its antibody antagonists in hemophagocytic lymphohistiocytosis
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摘要 噬血细胞性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)又称噬血细胞综合征(hemophagocytic syndrome,HPS),是多种病因引起的过度炎症综合征。在HLH的发病机制中,γ干扰素(interferon-γ,IFN-γ)发挥了重要作用,抗IFN-γ单抗可用于HLH的治疗。该文就IFN-γ在HLH发生机制中的作用以及抗IFN-γ单抗对HLH的治疗研究予以综述。 Hemophagocytic lymphohistiocytosis(HLH),also known as hemophagocytic syndrome(HPS),is a hyperinflammatory syndrome caused by multiple etiologies.Interferon-γ(IFN-γ)has been reported to play a key role in the pathogenesis of HLH,and antibody against IFN-γ(emapalumab)can be used to treat HLH.Here,the findings on the role of IFN-γin pathogenesis and emapalumab in treatment of HLH are reviewed respectively.
作者 程芬芬(综述) 张蕊 王天有(审校) Cheng Fenfen;Zhang Rui;Wang Tianyou(National Center for Children′s Health Beijing Children′s Hospital,Capital Medical University Hematology Oncology Center Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics Key Laboratory of Major Diseases in Children,Ministry of Education Beijing 100045,China)
出处 《国际儿科学杂志》 2021年第3期145-149,共5页 International Journal of Pediatrics
基金 国家科技重大专项(2017ZX09304029004) 国家自然科学基金(81700186,81800189) 北京市医院管理局中心儿科学科协同发展中心专项经费资助(XTZD20180202)。
关键词 噬血细胞性淋巴组织细胞增生症 发病机制 Γ干扰素 依法妥珠单抗 治疗 Hemophagocytic lymphohistiocytosis Pathogenesis IFN-γ Emapalumab Treatment
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