Leukaemia of natural killer cell large granular lymphocyte type with HLA-DR-CD16-CD56bright+ phenotype

J Prieto1, E Ríos, A Parrado

  • 1Hospital Universitario Virgen del Rocío, Servicio Andaluz de Salud, Sevilla, Spain.

Insights

This study reports a rare case of natural killer/large granular lymphocyte (NK/LGL) leukemia in a 45-year-old woman. The leukemia cells uniquely lacked HLA-DR expression, a characteristic not seen in previously documented cases.

Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Large granular lymphocyte (LGL) leukemia is a rare lymphoproliferative disorder.
  • Natural Killer (NK) cell lineage LGL leukemia is exceptionally uncommon.
  • This report details a unique case of acute NK/LGL leukemia.

Observation:

  • A 45-year-old woman presented with a rare form of leukemia.
  • Cytometric analysis revealed leukaemic blasts positive for CD2, CD38, and CD56, but negative for CD3, CD7, CD16, and notably, HLA-DR.
  • No T cell receptor or immunoglobulin gene rearrangements or chromosomal abnormalities were detected.

Findings:

  • The leukaemic blasts exhibited natural killer (NK) cell cytotoxicity.
  • A key finding was the absence of HLA-DR expression on leukaemic cells, which is highly unusual for acute NK cell leukemias.
  • The patient did not respond to chemotherapy and unfortunately died within three months of diagnosis.

Implications:

  • The unique immunophenotype, particularly the lack of HLA-DR, may represent an early developmental stage of NK cell precursors.
  • This case expands the understanding of the spectrum of NK/LGL leukemia.
  • Further research into NK cell ontogeny and aberrant differentiation in leukemia is warranted.