[Large granular lymphocyte leukemia].
Large granular lymphocyte (LGL) leukemia is a clonal proliferation of cytotoxic cells, either CD3(+) (T-cell) or CD3(-) (natural killer, or NK). Both subtypes can manifest as indolent or aggressive disorders. T-LGL leukemia is associated with cytopenias and autoimmune diseases and most often has an indolent course and good prognosis. Rheumatoid arthritis and Felty syndrome are frequent. NK-LGL leukemias can be more aggressive. LGL expansion is currently hypothesized to be a virus (Ebstein Barr or human T-cell leukemia viruses) antigen-driven T-cell response that involves disruption of apoptosis. The diagnosis of T-LGL is suggested by flow cytometry and confirmed by T-cell receptor gene rearrangement studies. Clonality is difficult to determine in NK-LGL but use of monoclonal antibodies specific for killer cell immunoglobulin-like receptor (KIR) has improved this process. Treatment is required when T-LGL leukemia is associated with recurrent infections secondary to chronic neutropenia. Long-lasting remission can be obtained with immunosuppressive treatments such as methotrexate, cyclophosphamide, and cyclosporine A. NK-LGL leukemias may be more aggressive and refractory to conventional therapy.
Lazaro
Estibaliz
E
Service de médecine interne et maladies infectieuses, Centre François Magendie, Hôpital du Haut-Lévêque, Pessac. estilazaro@gmail.com
Caubet
Olivier
O
Menard
Fanny
F
Pellegrin
Jean-Luc
JL
Viallard
Jean-François
JF
D006801:Humans; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy