Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas

Expert Rev Hematol. 2019 Nov;12(11):927-935. doi: 10.1080/17474086.2019.1660640. Epub 2019 Sep 5.


Introduction: Natural killer (NK)/T-cell lymphomas are aggressive malignancies that present predominantly in nasal and adjacent sites (nasal subtype), occasionally in skin, gastrointestinal tract and other tissues (non-nasal), and rarely as disseminated disease with a leukemic phase (aggressive NK-cell leukemia, or leukemia/lymphoma, subtype).Areas covered: The diagnosis and treatment of NK/T-cell lymphoma are discussed, based on a PubMed literature search. The diagnostic criteria for NK/T-cell lymphoma are highlighted, followed by an update of the diagnostic and prognostic importance (on presentation, at interim and end-of-treatment) of plasma EBV DNA as a surrogate biomarker of lymphoma load. Prognostic models based on clinicopathologic features and EBV DNA load are discussed. For stage I/II NK/T-cell lymphomas, combined chemotherapy, and radiotherapy gives the best results, with their concomitant or sequential administration equally efficacious. For stage III/IV NK/T-cell lymphoma, chemotherapy is the mainstay of treatment. Conventional anthracycline-based regimens for B-cell lymphomas are ineffective. Recommended regimens combine L-asparaginase with other drugs not affected by P-glycoprotein. For relapsed/refractory patients, immune checkpoint blockade with antibodies against programmed cell death protein 1 has shown much promise.Expert opinion: Current strategies result in durable remissions in a significant proportion of NK/T-cell lymphomas. Immune checkpoint inhibition and other novel approaches are promising for relapsed/refractory cases.

Keywords: Epstein Barr virus; L-asparaginase; NK/T-cell lymphoma; hematopoietic stem cell transplantation; programmed cell death protein 1.

Publication types

  • Review

MeSH terms

  • Anthracyclines / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Asparaginase / therapeutic use
  • Humans
  • Lymphoma, Extranodal NK-T-Cell / diagnosis*
  • Lymphoma, Extranodal NK-T-Cell / drug therapy*
  • Models, Biological*
  • Neoplasm Staging
  • Prognosis


  • Anthracyclines
  • Asparaginase