Primary and secondary autoimmune neutropenia

Arthritis Res Ther. 2005;7(5):208-14. doi: 10.1186/ar1803. Epub 2005 Aug 31.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Antibodies, Antineutrophil Cytoplasmic / immunology
  • Antibody Specificity
  • Antigens, CD / immunology
  • Autoantigens / immunology
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / therapy
  • Felty Syndrome / complications
  • Female
  • GPI-Linked Proteins
  • Granulocyte Colony-Stimulating Factor / therapeutic use
  • Hematologic Diseases / blood
  • Hematologic Diseases / complications
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Infant
  • Isoantigens / immunology
  • Leukemia, Lymphoid / complications
  • Lupus Erythematosus, Systemic / complications
  • Male
  • Neutropenia / drug therapy
  • Neutropenia / etiology
  • Neutropenia / immunology*
  • Neutropenia / therapy
  • Prevalence
  • Receptors, IgG / immunology
  • Rheumatic Diseases / blood
  • Rheumatic Diseases / complications

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Antigens, CD
  • Autoantigens
  • FCGR3B protein, human
  • GPI-Linked Proteins
  • Immunoglobulins, Intravenous
  • Isoantigens
  • Receptors, IgG
  • neutrophil-specific antigen NA1, human
  • Granulocyte Colony-Stimulating Factor