4B00.00
category
Constitutional neutropaenia
Definition
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
(4B4Y)
Also indexed as
Autosomal dominant severe congenital neutropaeniaAutosomal recessive severe congenital neutropaenia due to G6PC3 deficiencyAutosomal recessive severe congenital neutropaenia due to JAGN1 deficiencyBenign ethnic neutropaeniaCSF3R-related severe congenital neutropaeniaCongenital agranulocytosisCongenital neutropaenia associated with syndromal featuresCongenital neutropaenia with maturation arrestCongenital neutropaenia with myelokathexisCongenital neutropaeniasConstitutional neutropaeniaConstitutional neutropaenia with extra-haematopoietic manifestationsCyclic haematopoiesisCyclic neutropaeniaDursun syndromeInfantile genetic agranulocytosisKostmann diseaseKostmann syndromePrimary immunodeficiency syndrome due to p14 deficiencyPrimary immunodeficiency syndrome with short staturePulmonary arterial hypertension - leukopenia - atrial septal defectSevere congenital neutropaeniaX-linked severe congenital neutropaeniacongenital agranulocytosis and neutropaeniacongenital leukopaeniacyclic agranulocytosisprimary neutropaeniasevere infantile genetic agranulocytosissevere infantile genetic neutropaenia