Prevention and management of infections in children with severe aplastic anemia

Abstract

Aplastic anemia (AA) is a rare condition in children. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice for severe idiopatic AA. Survival in severe AA has markedly improved in the past decades due to advances in HSCT, immunosuppressive and biologic drugs, as well as supportive care. Since bacterial and fungal infections are one of the principal causes of morbidity and mortality in AA, one of the main challenges is to prevent the appearance of infections associated with severe and prolonged neutropenia. Most guidelines of treatment and prophylaxis are based on expert recommendations. Given the lack of controlled studies in children with AA, most recommendations of empiric treatment rely on guidelines for febrile-neutropenia management in hemato-oncologycal patients. A great variability exists in the use of antimicrobials for primary prophylaxis among different institutions. Due to the fact that patients with severe and prolonged AA present high incidence of filamentous fungal infections, an adecuate antifungal prophylaxis is recommended. In the case of severe lymphopenia, prophylaxis against P jirovecii should also be considered. Recommendations in prophylaxis and early treatment of infections in severe pediatric AA are detailed.