Neutropenia in childhood

Mature neutrophil granulocytes (short form neutrophils) serve as the defence against bacterial infections. A decrease in neutrophil numbers, neutropenia, is mostly associated with an increased risk of infections. The most common type of neutropenia in infancy, autoimmune neutropenia (AIN), is an exception due to self-limitation and usually without severe infections in the course of the disease. In comparison, severe congenital neutropenia was not treatable up to the late 1980s and often had a fatal outcome without a bone marrow transplantation. In the meantime, numerous genetic defects were identified, which caused neutropenia either isolated or in association with multiorgan diseases and accompanied by an increased risk of leukemic transformation. Since the availability of the hematopoietic growth factor granulocyte colony-stimulating factor (G-CSF, filgrastim or lenograstim as active substances) in the late 1980s, the prognosis of affected patients has significantly improved.

Automated summary

Mature neutrophil granulocytes play a crucial role in defending against bacterial infections. While a decrease in neutrophil numbers, known as neutropenia, typically increases the risk of infections, autoimmune neutropenia in infancy tends to be self-limiting and does not usually lead to severe infections. Severe congenital neutropenia, on the other hand, was previously untreatable and often led to fatal outcomes before the introduction of hematopoietic growth factors in the late 1980s.