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Common variable immunodeficiency phenotype due to IKAROS functional haploinsufficiency

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Disease definition

A rare syndrome with combined immunodeficiency characterized by a variable clinical presentation ranging from asymptomatic individuals to potentially life-threatening, recurrent bacterial infections associated with progressive loss of serum immunoglobulins and B cells.

ORPHA:317473

Classification level: Disorder

Synonym(s):
  • CVID phenotype due to IKAROS functional haploinsufficiency
  • Common variable immunodeficiency phenotype due to IKZF1 functional haploinsufficiency

Source: PubMed ID 21548011 26981933 36433803

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: Adolescent, Adult, Childhood, Infancy, Neonatal

ICD-10: D81.8

ICD-11: 4A01.1Y

OMIM: 616873

UMLS: C5190826

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

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

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2020) - J Clin Immunol Logo ERN
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