NK/T-cell lymphomas

Best Pract Res Clin Haematol. 2019 Sep;32(3):253-261. doi: 10.1016/j.beha.2019.06.005. Epub 2019 Jun 12.


NK/T-cell lymphomas are extranodal EBV-related malignancies, mostly of NK-cell and occasionally of T-cell lineage. They are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nose, nasopharynx and the upper aerodigestive tract. Non-nasal NK/T-cell lymphomas involve the skin, gastrointestinal tract, testis and other sites. Disseminated NK/T-cell lymphoma involves multiple organs, and may present with a leukemic phase. Initial evaluation requires positron emission tomography computed tomography (PET/CT) and quantification of circulating EBV DNA. Radiotherapy alone is inadequate with frequent relapses. Anthracycline-containing regimens are ineffective. Regimens incorporating asparaginase are currently the standard. For stage I/II disease, combined chemotherapy and radiotherapy is recommended. For stage III/IV disease, asparaginase-containing regimens are needed. Autologous hematopoietic stem cell transplantation (HSCT) is of limited efficacy, whereas allogeneic HSCT may be useful in patients with stage III/IV and relapsed diseases. Immunotherapy with antibodies against CD30, programmed cell death protein 1 and CD38 is promising.

Keywords: Asparaginase; EBV; Immunotherapy; NK/T-cell lymphoma; Nasal; Non-nasal; PD1; PET/CT.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents, Immunological / therapeutic use*
  • Asparaginase / therapeutic use*
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Lymphoma, Extranodal NK-T-Cell* / diagnostic imaging
  • Lymphoma, Extranodal NK-T-Cell* / immunology
  • Lymphoma, Extranodal NK-T-Cell* / pathology
  • Lymphoma, Extranodal NK-T-Cell* / therapy
  • Lymphoma, T-Cell, Peripheral* / diagnostic imaging
  • Lymphoma, T-Cell, Peripheral* / immunology
  • Lymphoma, T-Cell, Peripheral* / pathology
  • Lymphoma, T-Cell, Peripheral* / therapy
  • Positron Emission Tomography Computed Tomography*
  • Transplantation, Autologous


  • Antineoplastic Agents, Immunological
  • Asparaginase