This rare congenital disorder is characterized by regular oscillations of blood neutrophil count, usually with a periodicity of 14 to 21 days. Most cases occur sporadically, although a third have an autosomal dominant pattern of inheritance. The onset of neutropenia is usually in childhood when patients present with recurrent infections. Diagnosis may be difficult unless serial blood counts are performed. Treatment with granulocyte colony-stimulating factor shortens the period of neutropenia sufficiently to avoid infection.
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