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 |
| 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.