Abstract

Extra-nodal natural killer/T cell lymphoma, nasal type (ENKTL) is a rare subtype of non-Hodgkin lymphoma (NHL), characterized by high invasiveness, rapid progression and short survival. Currently, the pathogenesis of ENKTL is not fully understood and it may be related to Epstein-Barr virus (EBV) infection and genetic abnormality. In recent years, non-anthracycline drugs chemotherapy regimens based on L-asparaginase have improved the clinical outcomes of patients with ENKTL, but their overall prognosis are still poor, especially in patients with late and relapsed/refractory ENKTL. In order to better formulate effective treatment strategies and improve the prognosis of ENKTL, it is very necessary to put forward new therapeutic ideas, which mainly depends on the in-depth understanding of its pathogenesis. Therefore, this article reviews the research progress of pathogenesis of ENKTL. Key words: Lymphoma, extranodal NK-T-cell; Epstein-Barr virus; Genes; Signal pathways; Non-coding RNA

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