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Epstein\u2013Barr virus (EBV) infection in lymphoma cells is universal. Predominantly extranodal, NK\/T-cell lymphomas are divided clinically into nasal (involving the nose and upper aerodigestive tract), non-nasal (involving the skin, gastrointestinal tract, testes, and other organs), and aggressive leukaemia\/lymphoma (involving the marrow and multiple organs) subtypes. Initial assessment should include imaging with positron emission tomography computed tomography (PET\/CT), quantification of plasma EBV DNA as a surrogate marker of lymphoma load, and bone marrow examination with in situ hybridization for EBV-encoded small RNA. Prognostication can be based on presentation parameters (age, stage, lymph node involvement, clinical subtypes, and EBV DNA), which represent patient factors and lymphoma load; and dynamic parameters during treatment (serial plasma EBV DNA and interim\/end-of-treatment PET\/CT), which reflect response to therapy. Therapeutic goals are to achieve undetectable plasma EBV DNA and normal PET\/CT (Deauville score\u2009\u2264\u20093). NK\/T-cell lymphomas express the multidrug resistance phenotype, rendering anthracycline-containing regimens ineffective. Stage I\/II nasal cases are treated with non-anthracycline asparaginase-based regimens plus sequential\/concurrent radiotherapy. Stage III\/IV nasal, and non-nasal and aggressive leukaemia\/lymphoma cases are treated with asparaginase-containing regimens and consolidated by allogeneic haematopoietic stem cell transplantation (HSCT) in suitable patients. Autologous HSCT does not improve outcome. In relapsed\/refractory cases, novel approaches comprise immune checkpoint blockade of PD1\/PD-L1, EBV-specific cytotoxic T-cells, monoclonal antibodies, and histone deacetylase inhibitors. Future strategies may include inhibition of signalling pathways and driver mutations, and immunotherapy targeting the lymphoma and its microenvironment.<\/jats:p>","DOI":"10.1186\/s13045-022-01293-5","type":"journal-article","created":{"date-parts":[[2022,6,3]],"date-time":"2022-06-03T06:03:45Z","timestamp":1654236225000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":153,"title":["How we treat NK\/T-cell lymphomas"],"prefix":"10.1186","volume":"15","author":[{"given":"Eric","family":"Tse","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei-Li","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Xiong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yok-Lam","family":"Kwong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,6,3]]},"reference":[{"issue":"4","key":"1293_CR1","doi-asserted-by":"publisher","first-page":"1322","DOI":"10.1200\/JCO.1999.17.4.1322","volume":"17","author":"CS Chim","year":"1999","unstructured":"Chim CS, Ooi GC, Shek TW, Liang R, Kwong YL. 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