NK-cell lymphomas and leukemias: a spectrum of tumors with variable manifestations and immunophenotype

Am J Clin Pathol. 2007 Jun;127(6):860-8. doi: 10.1309/2F39NX1AL3L54WU8.

Abstract

Natural killer (NK) cells are lymphocytes that have some phenotypic and functional similarities to cytotoxic T cells but do not express the T-cell receptor complex. NK-cell malignancies may be localized or disseminated at initial examination, and most behave aggressively. The variable presentation of NK-cell lymphomas and leukemias suggests that they represent a spectrum of disease, with Epstein-Barr virus (EBV) implicated in the pathogenesis of most cases. Using cases presented in Session 10 of the 2005 Society for Hematopathology/European Association for Haematopathology Workshop on T-cell and NK-cell malignancies, we discuss outstanding issues in the classification and diagnosis of NK-cell malignancies. These difficulties are related to unusual sites of manifestation, atypical immunophenotypic features, and EBV+ T-cell tumors that resemble classical extranodal NK/T-cell lymphoma, nasal-type (EN-NK/T-NT). Although some of these cases can be grouped into ENNK/T-NT, classification of tumors that have atypical or discordant features will remain controversial, particularly when EBV is absent.

Publication types

  • Congress

MeSH terms

  • Epstein-Barr Virus Infections / complications
  • Epstein-Barr Virus Infections / immunology
  • Epstein-Barr Virus Infections / pathology
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / isolation & purification
  • Herpesvirus 4, Human / pathogenicity
  • Humans
  • Immunophenotyping
  • Killer Cells, Natural / pathology*
  • Leukemia / immunology
  • Leukemia / pathology*
  • Leukemia / virology
  • Lymphoma, Non-Hodgkin / immunology
  • Lymphoma, Non-Hodgkin / pathology*
  • Lymphoma, Non-Hodgkin / virology
  • RNA, Viral / isolation & purification
  • T-Lymphocytes / pathology*

Substances

  • RNA, Viral