1. This is the most common form of neutropenia in infants under 4 years of age. The rate of infection decreases with age.
2. Ninety percent of cases occur before 14 months of age.
3. The clinical course is variable, ranging from benign to life threatening. Patients with an ANC greater than 400/mm3 do not have a higher incidence of infection than normal children. Day-to-day variations occur in the white cell counts.
The ANC is usually less than 500/mm3. The bone marrow shows myeloid hyperpla-sia with a reduction in mature neutrophil precursors (maturation arrest).
Treatment includes supportive care, antibiotics, and G-CSF, if indicated.
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