Constitutional neutropaenia

Description

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.

Index terms

Constitutional neutropaenia
Congenital neutropaenias
congenital agranulocytosis and neutropaenia

Show allHide

congenital leukopaenia
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
Congenital agranulocytosis

Excludes

LD27.0Y – Cartilage-hair hypoplasia

Exclusions from above levels

MA16.10 – Decreased white blood cell count
02 – Neoplasms
18 – Complications of pregnancy, childbirth and the puerperium

Show allHide

20 – Developmental anomalies

Sibling codes

4B00.01 – Acquired neutropaenia
4B00.0Z – Neutropaenia, unspecified

Foundation entity

Constitutional neutropaenia – http://id.who.int/icd/entity/87096615

Part of: 4B00.0: Neutropaenia › 4B00: Disorders of neutrophil number › 04: Diseases of the immune system