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Natural killer cell leukemia. An autopsy case

Y Yatabe1, N Mori, N Hirabayashi

  • 1First Department of Pathology, Nagoya University School of Medicine, Japan.

Archives of Pathology & Laboratory Medicine
|December 1, 1994
PubMed

Insights

This autopsy case details natural killer cell leukemia, a rare aggressive cancer. Findings show leukemic cells infiltrating organs, distinct from other large granular lymphocytic leukemias.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Natural killer (NK) cell leukemia is a rare and aggressive hematologic malignancy.
  • Distinguishing NK cell leukemia from other large granular lymphocyte (LGL) disorders is crucial for accurate diagnosis and treatment.

Observation:

  • The autopsy case presented leukemic cells with morphology of large granular lymphocytes.
  • Immunophenotypic analysis revealed a restricted NK cell phenotype (CD2+, CD38+, CD56+, HLA-DR+).
  • Immunogenotypic analysis showed germline configurations of TCR-beta, TCR-gamma, and JH genes, supporting a non-T/non-B cell lineage.

Findings:

  • The clinicopathologic features align with the definition of natural killer cell leukemia.
  • Autopsy revealed extensive leukemic cell infiltration and architectural destruction across multiple organs, correlating with an aggressive clinical course.
  • Hemophagocytic histiocytosis and lack of chronic inflammatory changes differed from CD3+ LGL leukemia.

Implications:

  • This case contributes to understanding the clinicopathologic spectrum of natural killer cell leukemia.
  • The findings highlight the importance of comprehensive immunophenotypic and immunogenotypic analysis for diagnosing rare leukemias.
  • The distinct features observed differentiate this entity from other LGL leukemias, aiding in differential diagnosis.

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