[Congenital and acquired neutropenia in children]

Neutropenia is a common feature in pediatric pratice. Besides the particular etiological aspects in the newborn, neutropenia in a child may be acquired, part of a more complex genetic disease, or an isolated inborn disorder. Primary acquired neutropenia, also called benign chronic neutropenia, is the most frequent cause of chronic neutropenia in children. Infantile agranulocytosis is the main primary congenital neutropenia. Its underlying mechanisms are still unknown. Prevention of repeated infections is the main issue in the management of such patients. This implies careful individual assessment for each patient. Management includes first prophylactic antibiotherapy using trimethoprim-sulfamethoxazole and secondly hematological growth factors (mainly G-CSF). Long-term G-CSF therapy results in a rise in the absolute neutrophil count, a reduction in the rate of infection and an improvement in the patient's quality of life. Severe side effects, including osteoporosis, vasculitis, myelodysplasia or leukemia have been occasionally reported in certain subsets of patients. These features are currently being prospectively assessed in an international register. Use of hematological growth factors must be evaluated for each individual case.