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Related Experiment Videos

Blastic natural killer cell leukemia/lymphoma: a clinicopathologic study

J A DiGiuseppe1, D C Louie, J E Williams

  • 1Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287-6417, USA.

The American Journal of Surgical Pathology
|October 23, 1997
PubMed
Summary

Blastic NK-cell leukemia/lymphoma (NKL/L) is a distinct, aggressive malignancy affecting elderly patients. This study defines its unique clinical, pathological, and immunophenotypic features, often presenting in the skin and progressing to leukemia.

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • The classification of natural killer (NK)-cell and NK-like T-cell malignancies is evolving.
  • Blastic, blastoid, or monomorphic NK-cell leukemia/lymphoma (NKL/L) has been anecdotally described but not systematically defined.

Observation:

  • Four elderly patients (58-82 years) presented with cutaneous plaques, adenopathy, and marrow involvement.
  • Biopsies revealed atypical lymphoid infiltrates with blastic cytologic features in skin, lymph nodes, and marrow.
  • Tumor cells lacked conspicuous azurophilic granules, differing from typical NK disorders.

Findings:

  • Immunophenotyping showed intermediate CD45, HLA-DR, CD2, CD4, and CD56 positivity, with negative CD3 and CD5.
  • CD7 was positive in two cases, and CD43 positivity was noted in formalin-fixed tissue.

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  • Epstein-Barr viral RNA was negative in all tested cases.

    • Implications:

    • Blastic NKL/L represents a distinct clinicopathologic entity characterized by blastic NK cells.
    • The disease affects elderly individuals, has an aggressive clinical course, and can evolve into overt leukemia.
    • This systematic definition aids in diagnosing and understanding this rare NK-cell malignancy.