Autoimmune neutropenia (AIN) of infancy is an underreported disease because of its benign nature. In addition, the relative unavailability of neutrophil antibody testing prevents confirmation of suspected diagnoses. Small children do not have life-threatening infections, do not have associated diseases, respond to i.v. gamma globulin with increased neutrophil counts, and tend to improve on their own. Older children with AIN have associated diseases such as hepatitis or Hodgkin's disease, often have other immune cytopenias, and respond poorly to any therapy. Immune deficiency states, especially those of the humoral arms, are associated with development of AIN.
