[Large granular lymphocyte leukemia: clinical and pathogenic aspects].
Large granular lymphocyte leukemia (LGL) is a hematologic disorder characterized by a monoclonal expansion of large lymphocytes containing azurophilic granules with a T CD3(+)CD57(+) or Natural Killer (NK) CD3(-)CD56(+) phenotype. The World Health Organization (WHO) classification identifies three entities: the T LGL, the chronic lymphoproliferative disorder of NK-cells, and the aggressive NK-cell leukemia. T LGL and chronic lymphoproliferative disorder of NK-cells are indolent diseases frequently associated with cytopenias and a wide spectrum of auto-immune manifestations. Neutropenia can lead to recurrent bacterial infections, which represent an indication of initiating a treatment in most of the cases. Immunosuppressive therapies are usually used in this context. In contrast, aggressive NK-cell leukemia follows a fulminant course with a poor prognosis because patients are refractory to most of the treatments. There is now a considerable interest in the pathophysiology of the disease with the perspective of new therapeutic options.
Service de médecine interne, hôpital du Haut Lévêque, avenue Magellan, 33604 Pessac, France. estibaliz.lazaro@chu-bordeaux.fr
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Lazaro
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Duffau
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Chaigne Delalande
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Greib
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Pellegrin
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Viallard
D003937:Diagnosis, Differential; D005258:Felty Syndrome / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D015994:Incidence; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy
Cytopenia; Cytopénie; Felty's syndrome; Large granular lymphocytes; Leucémie; Leukemia; Lymphocytes granuleux; Neutropenia; Neutropénie; Polyarthrite rhumatoïde; Rheumatoid arthritis; Syndrome de Felty