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

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

2018

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

A 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.

Keywords:

Virology
Cancer cell
Medicine
T-cell lymphoma
Mutation
Immunology

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