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Natural killer cell neoplasms.

Eric Tse1, Raymond H S Liang

  • 1University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong.

Clinical Lymphoma
|January 8, 2005
PubMed
Summary
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Natural killer (NK) cell neoplasms are rare lymphoid cancers. These aggressive diseases, including extranodal NK cell lymphoma, nasal-type, and aggressive NK cell leukemia, often involve extranodal sites and show poor treatment response.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Lymphoid neoplasms originating from natural killer (NK) cells are uncommon but distinct clinicopathologic entities.
  • The latest World Health Organization classification recognizes three types: extranodal NK cell lymphoma, nasal-type; aggressive NK cell leukemia; and blastic NK cell lymphoma.

Observation:

  • NK cell tumors express the CD56 marker but lack surface CD3 and T-cell receptor gene rearrangements, differentiating them from T-lymphoid neoplasms.
  • Epstein-Barr virus (EBV) is strongly associated with these neoplasms, except for blastic NK cell lymphoma.
  • Extranodal involvement, particularly in the nasal cavity, skin, and gastrointestinal tract, is common.

Findings:

  • All three recognized NK cell neoplasms exhibit an aggressive clinical course.

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  • These cancers generally show a poor response to conventional treatments.
  • Localized disease has demonstrated an initial positive response to combined radiation and chemotherapy.

    • Implications:

    • Further research into NK cell biology and neoplasm pathology is crucial.
    • Understanding these mechanisms may lead to the development of novel and more effective therapeutic strategies.
    • Optimizing treatment modalities for NK cell neoplasms remains an ongoing challenge.