[Large granular lymphocyte proliferations. Clinical and pathogenic aspects].
BACKGROUND
The clinical course, biological features, and recent data on the pathogenesis of large granular lymphocyte (LGL) leukemia are reviewed.


BACKGROUND
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).


CONCLUSIONS
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.
Hamidou
M
M
Service de médecine interne A, Hôtel-Dieu, CHU, place Alexis-Ricordeau, 44035 Nantes, France. mohamed.hamidou@chu-nantes.fr
Lamy
T
T
D001327:Autoimmune Diseases / Q000150:complications; D015139:Blotting, Southern; D003520:Cyclophosphamide / Q000627:therapeutic use; D016207:Cytokines / Q000276:immunology; D005602:France / Q000453:epidemiology; D015490:HTLV-I Infections / Q000150:complications; D006801:Humans; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007945:Leukemia, Lymphoid / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D008727:Methotrexate / Q000627:therapeutic use; D009503:Neutropenia / Q000209:etiology; D016133:Polymerase Chain Reaction; D011379:Prognosis; D012042:Registries; D013163:Splenomegaly; D019014:fas Receptor / Q000276:immunology
D016207:Cytokines; D007166:Immunosuppressive Agents; D019014:fas Receptor; D003520:Cyclophosphamide; D008727:Methotrexate