Antineutrophil antibodies are well recognized causes of neutropenia, producing both quantitative and qualitative defects in neutrophils and increased risk for infection. In primary autoimmune neutropenia (AIN) of infancy, a moderate to severe neutropenia is the sole abnormality; it is rarely associated with serious infections and exhibits a self-limited course. Chronic idiopathic neutropenia of adults is characterized by occurrence in late childhood or adulthood, greater prevalence among females than among males, and rare spontaneous remission. Secondary AIN is more commonly seen in adults and underlying causes include collagen disorders, drugs, viruses and lymphoproliferative disorders. In most patients with AIN, antibodies recognize antigens located on the IgG Fc receptor type 3b but other target antigens have been recently identified in secondary AIN. Granulocyte colony-stimulating factor is a proven treatment in patients with AIN of all types and is now preferred to other possible therapies. (copyright) 2005 BioMed Central Ltd.
|Titolo:||Primary and secondary autoimmune neutropenia|
CAPSONI, FRANCO (Primo)
|Parole Chiave:||Felty syndrome; autoimmune disease; collagen disease; cytotoxic T lymphocyte; diagnostic test; disease association; disease classification; disease severity; dose response; human; immunofluorescence test; lymphoproliferative disease; neutropenia; neutrophil chemotaxis; nonhuman; pathophysiology; qualitative analysis; quantitative analysis; review; risk factor; systemic lupus erythematosus; Fc receptor; autoantibody; corticosteroid; cyclosporin A; fludarabine; gold derivative; granulocyte antibody; granulocyte colony stimulating factor; immunoglobulin G; leflunomide; methotrexate; prednisone; propylthiouracil; rituximab|
|Settore Scientifico Disciplinare:||Settore MED/16 - Reumatologia|
|Data di pubblicazione:||ott-2005|
|Digital Object Identifier (DOI):||10.1186/ar1803|
|Appare nelle tipologie:||01 - Articolo su periodico|