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Updated: Aug 8, 2026

Flow Cytometry to Estimate Leukemia Stem Cells in Primary Acute Myeloid Leukemia and in Patient-derived-xenografts, at Diagnosis and Follow Up
Published on: March 26, 2018
Leukaemia of natural killer cell large granular lymphocyte type with HLA-DR-CD16-CD56bright+ phenotype
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.
Abstract:
The case is reported of a 45 year old woman with the rare leukaemia of natural killer cell large granular lymphocyte (NK/ LGL) type. Cytometric analysis of leukaemic blasts showed that they were positive for CD2, CD38, and CD56 antigens but negative for a series of antigens including CD3, CD7, CD16, and HLA-DR. Rearrangements of the beta T cell receptor, and heavy and kappa immunoglobulin genes were not detected and neither were chromosomal abnormalities. Leukaemic blasts developed NK cytotoxicity. The patient failed to respond to aggressive chemotherapy and died three months after diagnosis. The lack of expression of HLA-DR is an extraordinary characteristic of this case, as all cases of acute NK cell leukaemias described to date expressed HLA-DR. The immunophenotype observed in the NK cell leukaemic blasts may represent the counterpart of a hypothetical normal cell precursor in an early stage of ontogenic NK cell development.
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