infantile genetic agranulocytosis

An inherited disorder in which there is a lower-than-normal number of neutrophils (a type of white blood cell that is important in fighting infections). Infants with the disorder get infections caused by bacteria, and are at an increased risk of acute myelogenous leukemia (AML) or myelodysplasia (a bone marrow disorder). Also called congenital neutropenia, genetic infantile agranulocytosis, Kostmann disease, Kostmann neutropenia, and Kostmann syndrome.