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[Large granular lymphocyte leukemia]

M Mizuki1, S Tagawa, M Shibano

  • 1Department of Hematology and Oncology, Osaka University Medical School, Suita.

Rinsho Byori. the Japanese Journal of Clinical Pathology
|October 1, 1996
PubMed
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Large granular lymphocyte leukemia (LGLL) involves clonal LGL proliferation. Studies reveal LGLL cells infiltrate organs, CD95 influences slow growth, and DP-T-LGLL may arise from IL-4 activated T cells.

Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Large granular lymphocyte leukemia (LGLL) is a clonal proliferation of large granular lymphocytes (LGLs) in peripheral blood.
  • Chronic LGLL often presents indolently, posing diagnostic challenges due to lack of distinct organomegaly or monoclonality evidence.

Observation:

  • Immunohistological studies in a persistent NK lymphocytosis patient revealed multi-organ infiltration upon autopsy, despite no apparent organomegaly during a three-year observation period.
  • Both T- and NK-LGLL cells expressed CD95, an apoptosis-related protein, with anti-CD95 suppressing IL-2 or anti-CD3 induced proliferation.
  • CD4+CD8+ double positive (DP) LGLL cases, rare in LGLL, exhibited alpha alpha CD8 subunit type, lacked RAG-1 expression, and secreted IL-4, characteristic of peripheral T cells.

Findings:

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  • Chronic LGLL cells demonstrate infiltrative capacity, characteristic of malignant cells.
  • The CD95-CD95 ligand system appears to regulate the slow cell growth typical of chronic LGLL.
  • DP-T-LGLL likely represents an expansion of a rare peripheral DP-T cell subset, potentially originating from IL-4 activated CD4+ T cells.

Implications:

  • Findings highlight the infiltrative potential of chronic LGLL cells, necessitating careful monitoring.
  • Understanding the CD95 pathway's role in LGLL proliferation offers potential therapeutic targets.
  • Identifying the origin of DP-T-LGLL provides insights into T-cell subsets and their role in leukemia development.