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X-linked severe congenital neutropenia

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Disease definition

X-linked severe congenital neutropenia is an immunodeficiency syndrome characterized by recurrent major bacterial infections, severe congenital neutropenia, and monocytopenia. It has been described in five males spanning three generations of one family. It is transmitted as an X-linked recessive trait and is caused by mutations in the WAS gene, encoding the WASP protein.

ORPHA:86788

Classification level: Disorder

Prevalence: <1 / 1 000 000

Inheritance: X-linked recessive

Age of onset: Infancy, Neonatal

ICD-10: D70

ICD-11: 4B00.00

OMIM: 300299

UMLS: C1845987

MeSH: C564539

GARD: 3981

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 (2024) - 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.