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Autosomal dominant severe congenital neutropenia

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Disease definition

A rare primary immunodeficiency disorder characterized by autosomal dominant inheritance, absolute neutrophil counts below 0.5x10E9/L in the peripheral blood (on three separate occasions over a six month period), granulopoiesis maturation arrest at the promyelocyte/myelocyte stage and early-onset, severe, recurrent bacterial infections.

ORPHA:486

Classification level: Disorder

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: Infancy, Neonatal

ICD-10: D70

ICD-11: 4B00.00

OMIM: 619813 202700 257100 613107

UMLS: C4749612

GARD: 9558

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2019) - Socialstyrelsen
Guidelines
Emergency guidelines
Français (2009.pdf) - Orphanet Urgences
Deutsch (2009.pdf) - Orphanet Urgences
Español (2009.pdf) - Orphanet Urgences
Italiano (2012.pdf) - Orphanet Urgences
Clinical practice guidelines
Disease review articles
Review article
English (2011) - Orphanet J Rare Dis
Clinical genetics review
English (2018) - GeneReviews
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.