Primary and secondary autoimmune neutropenia.
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.
Capsoni
Franco
F
Rheumatology Unit, Istituto Ortopedico Galeazzi, University of Milan, Milan, Italy. franco.capsoni@unim.it
Sarzi-Puttini
Piercarlo
P
Zanella
Alberto
A
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D000918:Antibody Specificity; D015703:Antigens, CD / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000628:therapy; D005258:Felty Syndrome / Q000150:complications; D005260:Female; D058851:GPI-Linked Proteins; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006402:Hematologic Diseases / Q000097:blood / Q000150:complications; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D007519:Isoantigens / Q000276:immunology; D007945:Leukemia, Lymphoid / Q000150:complications; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008297:Male; D009503:Neutropenia / Q000188:drug therapy / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D015995:Prevalence; D017452:Receptors, IgG / Q000276:immunology; D012216:Rheumatic Diseases / Q000097:blood / Q000150:complications
D019268:Antibodies, Antineutrophil Cytoplasmic; D015703:Antigens, CD; D001324:Autoantigens; C428200:FCGR3B protein, human; D058851:GPI-Linked Proteins; D016756:Immunoglobulins, Intravenous; D007519:Isoantigens; D017452:Receptors, IgG; C067704:neutrophil-specific antigen NA1, human; D016179:Granulocyte Colony-Stimulating Factor