Table 1.

Common features of chronic autoimmune/benign/idiopathic neutropenia of childhood

Clinical features	Median age of diagnosis is 8-11 months of age (range 3-38 mo)
	Few and minor infections (mostly upper respiratory)
	Occasional gingivitis
	Rare serious or invasive infections (eg, pneumonia), usually in young infants
Laboratory features	Median ANC at time of diagnosis ≈200 cells/µL (range 0-500)
	ANC often rises at times of stress or bacterial infection
	Hemoglobin, platelet count generally normal
	Bone marrow (if performed) shows normal to increased myelopoiesis, sometimes with a decrease in mature neutrophils
	Antineutrophil antibodies sometimes detected but not predictive of course
Therapy	Antibiotics for acute bacterial infection
	Prophylactic antibiotics (eg, trimethoprim/sulfamethoxazole) may be helpful in some patients with recurrent otitis media, but can exacerbate neutropenia
	G-CSF only in the event of serious infection (consider alternative diagnoses!) or to improve quality of life in case of very frequent precautionary emergency department visits or admissions
Prognosis	Recovery in almost all patients (median duration 20 mo, range 6-54 mo)
	No evident risk of recurrence

Adapted from Dinauer et al¹ with permission.