4B00.00 Constitutional neutropaenia
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
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
synonyms
- Constitutional neutropaenia
- congenital agranulocytosis and neutropaenia
- congenital leukopaenia
- Congenital neutropaenias
- primary neutropaenia
- Benign ethnic neutropaenia
- Severe congenital neutropaenia
- severe infantile genetic neutropaenia
- Autosomal dominant severe congenital neutropaenia
- Autosomal recessive severe congenital neutropaenia due to G6PC3 deficiency
- Dursun syndrome
- Pulmonary arterial hypertension - leukopenia - atrial septal defect
- Autosomal recessive severe congenital neutropaenia due to JAGN1 deficiency
- CSF3R-related severe congenital neutropaenia
- Kostmann syndrome
- Kostmann disease
- X-linked severe congenital neutropaenia
- Congenital neutropaenia with myelokathexis
- Congenital neutropaenia with maturation arrest
- Congenital neutropaenia associated with syndromal features
- Cyclic neutropaenia
- cyclic agranulocytosis
- Cyclic haematopoiesis
- Constitutional neutropaenia with extra-haematopoietic manifestations
- Primary immunodeficiency syndrome due to p14 deficiency
- Primary immunodeficiency syndrome with short stature
- Infantile genetic agranulocytosis
- severe infantile genetic agranulocytosis
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