4B00.00
Constitutional neutropaenia
Neutropenia constitucional
CategoryDefinition
This is a granulocyte disorder characterised by an abnormally low number of neutrophils. Neutrophils usually make up 50-70% of circulating white blood cells and serve as the primary defence against infections by destroying bacteria in the blood.
Exclusions
- Cartilage-hair hypoplasia
Index Terms
Constitutional neutropaeniacongenital agranulocytosis and neutropaeniacongenital leukopaeniaCongenital neutropaeniasprimary neutropaeniaBenign ethnic neutropaeniaSevere congenital neutropaeniasevere infantile genetic neutropaeniaAutosomal dominant severe congenital neutropaeniaAutosomal recessive severe congenital neutropaenia due to G6PC3 deficiencyDursun syndromePulmonary arterial hypertension - leukopenia - atrial septal defectAutosomal recessive severe congenital neutropaenia due to JAGN1 deficiencyCSF3R-related severe congenital neutropaeniaKostmann syndromeKostmann diseaseX-linked severe congenital neutropaeniaCongenital neutropaenia with myelokathexisCongenital neutropaenia with maturation arrestCongenital neutropaenia associated with syndromal featuresCyclic neutropaeniacyclic agranulocytosisCyclic haematopoiesisConstitutional neutropaenia with extra-haematopoietic manifestationsPrimary immunodeficiency syndrome due to p14 deficiencyPrimary immunodeficiency syndrome with short statureInfantile genetic agranulocytosissevere infantile genetic agranulocytosis