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

• Cartilage-hair hypoplasia  (LD27.0)

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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