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Monoclonal expansion of large granular lymphocytes with a CD4+ CD8dim+/- phenotype associated with hairy cell

P Airò1, G Rossi, F Facchetti

  • 1Servizio di Immunologia Clinica, Spedali Civili ed Università, Brescia, Italy.

Haematologica
|March 1, 1995
PubMed

Insights

Unusual lymphoid cell expansions, known as large granular lymphocytes (LGL), can be monoclonal. This case reveals a monoclonal LGL expansion alongside B-cell hairy cell leukemia, suggesting a potential link between these conditions.

Area of Science:

  • Immunology
  • Hematology
  • Oncology

Background:

  • Peripheral blood lymphoid cell expansions with a CD3+, CD4+, CD8dim+/-, CD11b+, CD57+ immunophenotype, often resembling large granular lymphocytes (LGL), have been recently identified.
  • These LGL expansions can be monoclonal or polyclonal, and their clinical significance remains largely undetermined, with no established association with hematological neoplasms.

Observation:

  • This report details a unique case involving a patient presenting with a monoclonal expansion of LGL.
  • Concurrently, the patient was diagnosed with B-cell-derived hairy cell leukemia.

Findings:

  • The study identified two distinct lymphoid clones within the patient: a monoclonal T-cell large granular lymphocyte (T-LGL) population in the peripheral blood and hairy cells in the bone marrow and kidney.
  • These two clones, T-LGL and hairy cell leukemia, were not found to be physically associated, indicating separate clonal origins or distributions.

Implications:

  • This case highlights a potential, previously undescribed association between monoclonal LGL expansions and hairy cell leukemia.
  • Further research is warranted to elucidate the biological relationship and clinical implications of co-occurring T-LGL expansions and B-cell malignancies.

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