Kostmann syndrome


A congenital (present at birth) condition in which there is a severe lack of neutrophils (a type of white blood cell that helps defend the body against bacteria, viruses and fungi). Children with this condition have frequent infections.

Kostmann syndrome is associated with an increased risk of acute myelogenous leukemia (AML) or bone marrow disorders.

Also called Kostmann disease, Kostmann neutropenia, congenital neutropenia and infantile genetic agranulocytosis.