[Autoimmune neutropenias].
Primary auto-immune neutropenia (AIN) is usually described in children. Secondary AIN occurs in collagen vascular diseases such as rheumatoid arthritis and (Felty's syndrome), Gougerot-Sjogren syndrome, and systemic lupus erythematosus. Some cases of other immune cytopenia (idiopathic thrombocytopenic purpura, Evans's syndrome) or lymphoproliferative disorders (large granular lymphocyte leukemia, malignant lymphoma) may be associated with AIN. Some cases of primary AIN occur, especially in children. The diagnosis of AIN depends on the demonstration of autoantibodies directed against neutrophil-specific antigens like CD16. The availability of granulocyte-colony stimulating factor for the treatment of AIN has been a major advance. In some cases, immunosuppressive therapy using prednisone, methotrexate, cyclosporine A must be added, especially in cases of secondary AIN.
Lamy
T
T
Service d'hématologie clinique CHU-Hôpital Pontchaillou 35033 Rennes. Thierry.Lamy@univ-rennes1.fr
D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009503:Neutropenia / Q000209:etiology / Q000276:immunology* / Q000628:therapy
D007166:Immunosuppressive Agents; D016179:Granulocyte Colony-Stimulating Factor