Recurrent ECSIT mutation encoding V140A triggers hyperinflammation and promotes hemophagocytic syndrome in extranodal NK/T cell lymphoma

Haijun Wen,Huajuan Ma,Qichun Cai,Suxia Lin,Xinxing Lei,Bin He,Sijin Wu,Zifeng Wang,Yan Gao,Wensheng Liu,Weiping Liu,Qian Tao,Zijie Long,Min Yan,Dali Li,Keith W. Kelley,Yongliang Yang,Huiqiang Huang,Quentin Liu

NATURE MEDICINE（2018）

引用 57|浏览33

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摘要

recurrent ECSIT mutation in individuals with extranodal natural killer/T cell lymphoma induces NFκB in cancer cells, leading to macrophage activation, and associates with progression to fatal hemophagocytic syndrome. NFκB-targeting therapy produced stable remission in two patients.

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关键词

Cancer genetics,T-cell lymphoma,Biomedicine,general,Cancer Research,Metabolic Diseases,Infectious Diseases,Molecular Medicine,Neurosciences

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