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About 85% of ENKTLs derive from NK cells and 15% from T\u2010cells. Various factors have been implicated in the development of ENKTL. Molecular pathogenesis of NK\/T\u2010cell lymphomas include mutations of genes, involving in the Janus Kinase\/signal transducer and activator of transcription pathway, RNA helicase family, epigenetic regulation, and tumor suppression. The relationship between ENKTL and human leukocyte antigen has been demonstrated. Radiotherapy plays a key role in the first\u2010line treatment of early\u2010stage. In stage III\/IV diseases, non\u2010anthracycline\u2010regimens\u2010containing L\u2010asparaginase are recommended. Although clinical remission after L\u2010asparaginase\u2010based combination therapy has been achieved in the majority of patients with advanced\u2010stage or relapsed\/refractory extranodal NK\/T\u2010cell lymphoma\u2010nasal type, the long\u2010term overall survival is still poor. Recently, immunotherapy and new therapeutic targets have gained much attention. In this article, we discuss the pathogenesis, diagnosis, prognostic models and management options of ENKTL.<\/jats:p>","DOI":"10.1002\/hon.2944","type":"journal-article","created":{"date-parts":[[2021,11,3]],"date-time":"2021-11-03T16:28:15Z","timestamp":1635956895000},"page":"221-229","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Review on natural killer\/T\u2010cell lymphoma"],"prefix":"10.1002","volume":"41","author":[{"given":"Xiaohua","family":"He","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine Sun Yat\u2010sen University Cancer Center  Guangzhou People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, State Key Laboratory of Oncology in South 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