Primary and secondary autoimmune neutropenia

Franco Capsoni, Piercarlo Sarzi-Puttini, Alberto Zanella

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)208-214
Number of pages7
JournalArthritis Research and Therapy
Issue number5
Publication statusPublished - Oct 2005

ASJC Scopus subject areas

  • Rheumatology


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