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Large granular lymphocytosis

R Zambello1, G Semenzato

  • 1Division of Hematology, Vicenza Hospital, Padua, Italy.

Haematologica
|November 27, 1998
PubMed
Summary
This summary is machine-generated.

Lymphoproliferative disease of granular lymphocytes (LDGL) is a spectrum of disorders from mild to aggressive. Diagnosis involves identifying chronically expanded granular lymphocytes, with treatment tailored to symptom severity.

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Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Granular lymphocytes (GL) increase is often reactive, but a specific disease, lymphoproliferative disease of GL (LDGL), is recognized.
  • LDGL presents a spectrum from asymptomatic lymphocytosis to aggressive, potentially fatal conditions.

Purpose of the Study:

  • To review the clinical, biological, and pathogenetic mechanisms of lymphoproliferative disease of granular lymphocytes (LDGL).
  • To discuss diagnostic criteria, immunologic and molecular evaluations, and therapeutic strategies for LDGL.

Main Methods:

  • Review of literature on over 500 reported LDGL patients.
  • Analysis of diagnostic criteria, immunologic classification (CD3+ vs. CD3-), and molecular evaluations.
  • Examination of clinical features, associated infections, and therapeutic approaches.

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Main Results:

  • LDGL is classified into CD3+ (more common, symptomatic in 50% with neutropenia, infections, anemia) and CD3- (15% of cases, often linked to viral infections).
  • T-cell receptor clonality is common in CD3+ LDGL and indicates an aggressive course when present in CD3- LDGL.
  • Cytokines like IL-2, IL-12, and IL-15 may play a role; combination therapy (methotrexate/steroids) is effective for symptomatic cases. Spontaneous regression occurs.

Conclusions:

  • LDGL is a recognized disorder with a wide clinical spectrum, requiring confirmation of chronically expanded GL subset for diagnosis.
  • Asymptomatic patients should have therapy delayed; combination therapy with methotrexate or cyclophosphamide and steroids is the preferred approach when treatment is necessary.