Neutropenia (In Infancy and Childhood)

Abstract

Neutropenia is defined as a decrease in the number of circulating neutrophils in the peripheral blood with absolute neutrophil count less than 1000–1500/μL. Chronic neutropenia in pediatric patients is divided into three groups. Extrinsic factors, such as antibodies, some drugs, and nutritional deficiencies, lead to excessive destruction of neutrophils. Autoimmune neutropenia is a benign form of neutropenia shown in infancy to early childhood. Spontaneous recovery of neutropenia usually occurs within a few months to a few years. Acquired disorders of myeloid and stem cells present hypoplasia of myeloid cells. Congenital neutropenia is intrinsic defects in granulocytes or their progenitors and includes a heterogenous group of disorders. More than ten responsible gene mutations have been identified in congenital neutropenia. Most common congenital neutropenia is due to the gene mutation of neutrophil elastase. The hallmark of profound neutropenia is increased susceptibility to bacterial infections, cutaneous cellulitis, deep tissue abscesses, pneumonia, and septicemia. Almost patients with congenital neutropenia have been responded to administration of G-CSF. However, long-term use of G-CSF has the risk of the development of MDS/AML, suggesting the necessity of the careful follow-up. Hematopoietic stem cell transplantation should be considered for the curable treatment in severe congenital neutropenia.