Autoimmune neutropenia AIN

Infant form of isolated autoimmune neutropenia occurs within 1st year of life; demonstrable autoantibodies. Not familial; girls>boys. Self-limiting and usually relatively benign. Therapy supportive. Steroids not usually needed and can increase infection risk. IVIg has been used.

Older children may get

• Isolated AI neutropenia.

• Neutropenia as part of Evans' syndrome (ffl p388).

• Neutropenia as part of multi-system AI disease—lupus erythematosus

• Immune neutropenia following allogeneic BMT.

• Felty's syndrome (rheumatoid arthritis with splenomegaly and hyper-splenic cytopenias—ffl p17) may also be associated with neutrophil autoantibodies.

• All are potentially more serious and complicated than the infant form and may require immunosuppression as well as supportive therapy.

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