Neutropenia in pediatric practice

Abstract

Neutropenia unrelated to chemotherapy toxicity occurs in a number of clinical settings. The most common conditions associated with neutropenia are those that are acquired, including viral infection, neutropenia associated with various medications, and immune neutropenia. Inherited neutropenias are rarer and often more profound. These disorders include the dominant or sporadic types of severe congenital neutropenia (often with mutations in the ELA2 gene), the recessive type or Kostmann syndrome, and the marrow failure syndromes such as Fanconi anemia. Cyclic neutropenia may be severe at the nadir of the cycle. Of particular concern is the occurrence of fever in conjunction with neutropenia. This combination creates a medical emergency that must be addressed with appropriate evaluation and prompt administration of antibiotics. The actual risk of severe infection and the likelihood of recovery depend not only on the level of the ANC, but on the duration of the neutropenia. If recovery from the neutropenia is not expected, as in severe congenital types, G-CSF administration may be indicated.