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Clonal disease of natural killer large granular lymphocytes in Taiwan

W C Chou1, I P Chiang, J L Tang

  • 1Department of Internal Medicine, National Taiwan University Hospital, Taipei.

British Journal of Haematology
|January 14, 1999
PubMed

Insights

Natural killer (NK) large granular lymphocyte (LGL) leukemia is a rare disorder. This study found Epstein-Barr virus (EBV) association and specific chromosomal abnormalities in aggressive NK-LGL leukemia cases.

Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Lymphoproliferative diseases of large granular lymphocytes (LDGL) can originate from T cells or natural killer (NK) cells.
  • Large granular lymphocyte (LGL) leukemia is defined by the clonal proliferation of LGL.
  • Natural killer (NK)-LGL leukemia is a rare entity with unclear pathogenesis.

Purpose of the Study:

  • To investigate the characteristics of patients with cytogenetically confirmed NK-LGL leukemia.
  • To explore the association with Epstein-Barr virus (EBV) and identify common chromosomal abnormalities.

Main Methods:

  • Retrospective analysis of six patients diagnosed with NK-LGL leukemia between 1991 and 1998.
  • Serological testing for EBV, detection of EBV RNA/DNA in LGL cells using in situ hybridization or Southern blot.
  • Cytogenetic analysis to identify chromosomal abnormalities.

Main Results:

  • All six patients were seropositive for EBV, with EBV detected in LGL cells of four patients.
  • The disease often presented an aggressive clinical course, with five of the six patients dying.
  • Common cytogenetic findings included duplication of 1q, rearrangement at 3q, and loss of Y, 13, or 10 chromosomes.

Conclusions:

  • NK-LGL leukemia is frequently associated with Epstein-Barr virus.
  • Recurrent chromosomal aberrations are observed in NK-LGL leukemia, suggesting their role in disease development and progression.
  • Further research is needed to understand the implications of these cytogenetic abnormalities.

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