[Large granular lymphocyte proliferations. Clinical and pathogenic aspects].

Abstract

INTRODUCTION:

The clinical course, biological features, and recent data on the pathogenesis of large granular lymphocyte (LGL) leukemia are reviewed.

CURRENT KNOWLEDGE AND KEY POINTS:

Clonal diseases of LGL disorders can arise from a CD3+, CD57+ T-cell lineage, which are the most frequent, or from a CD3-, CD56+ NK-cell lineage. The diagnosis of LGL leukemia is suspected on the basis of a persistent excess of LGL, usually with neutropenia and splenomegaly. It is assessed by immunophenotypic and molecular studies of T-cell receptor clonality (southern blot, PCR). Association with autoimmune diseases (rheumatoid arthritis, erythroblastopenia, etc.) is a main feature of chronic LGL proliferation. Questions about a viral agent (HTLV1?), facilitation of clonal expansion by cytokines (IL-12, IL-15), and the defective Fas apoptotic pathway are discussed. Treatment of symptomatic LGL proliferations is based on immunosuppressive agents (principally methotrexate and cyclophosphamide).

FUTURE PROSPECT AND PROJECTS:

The epidemiology, prognosis factors, therapeutics and the pathogenesis of LGL leukemia are unknown. We proposed the creation of a French register of LGL expansions to explore these different aspects.

• Authors: M Hamidou, T Lamy
• Journal: La Revue de medecine interne (Rev Med Interne) Vol. 22 Issue 5 Pg. 452-9 (May 2001) ISSN: 0248-8663 [Print] France
• Vernacular Title: Proliférations chroniques à grands lymphocytes granuleux. Aspects cliniques et pathogéniques.
• PMID: 11402516 (Publication Type: Journal Article, Review)

Chemical References

• Cytokines
• Immunosuppressive Agents
• fas Receptor
• Cyclophosphamide
• Methotrexate

Topics

• Autoimmune Diseases (complications)
• Blotting, Southern
• Cyclophosphamide (therapeutic use)
• Cytokines (immunology)
• France (epidemiology)
• HTLV-I Infections (complications)
• Humans
• Immunophenotyping
• Immunosuppressive Agents (therapeutic use)
• Leukemia, Lymphoid (blood, diagnosis, drug therapy, epidemiology, etiology)
• Methotrexate (therapeutic use)
• Neutropenia (etiology)
• Polymerase Chain Reaction
• Prognosis
• Registries
• Splenomegaly
• fas Receptor (immunology)