History

Most children with autoimmune neutropenia receive initial medical attention because of the occurrence of a febrile illness during the last 6 months of infancy. Such illnesses include the following:

A simple febrile illness
Acute otitis media
Upper respiratory tract infection
Pneumonia
Gastroenteritis
Tonsillitis/pharyngitis
Skin infection

For primary AIN, a family history of neutropenia, leukopenia, and consanguinity is absent. This is in contrast to congenital neutropenia or ethnic (or familial) neutropenia. If a complete blood count (CBC) is performed earlier in the child's life, it is usually within the reference range.

Children with autoimmune neutropenia may have a history of frequent upper respiratory infections or of other infections listed above. However, serious, life-threatening infections, such as sepsis, osteomyelitis, deep tissue cellulitis/abscess, or bacterial meningitis, are very rare. Some patients are asymptomatic. A CBC performed for an unrelated reason, such as a routine CBC, may reveal neutropenia.

A family history of neutropenia or consanguinity, a history of serious past infections, or documentation of past neutropenia in the child significantly favors the diagnosis of congenital neutropenia or a secondary neutropenia including immunodeficiency, rather than acquired AIN, and indicates a worse prognosis.^[32]

Physical Examination

Physical examination may reveal signs of a local infection, including mouth ulcer, gingivitis, upper respiratory tract infections, impetigo, otitis media, pneumonia, and, very rarely, cellulitis, abscesses, or sepsis.

Many children may simply present with fever without any focal infection or they may physically be entirely normal. Patients generally do not exhibit growth failure or chronic illness.

Differential Diagnoses

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Media Gallery

A case of secondary autoimmune neutropenia. This patient presented with recurrent otitis and areas of cellulitis in the diaper area. Pseudomonas aeruginosa and Staphylococcus aureus were isolated from the skin lesions. Autoimmune hemolytic anemia and autoimmune neutropenia were confirmed based on the presence of autoantibodies. The patient has a mutation on exon 15, A504T, which changed an asparagine residue to a valine residue.

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