Other or unspecified common variable immunodeficiency

Description

obsolete_common variable immunodeficiency: ['A hypogammaglobulinemia that is results in insufficient production of antibodies needed to respond to exposure of pathogens and is characterized by low Ig levels with phenotypically normal B cells that can proliferate but do not develop into Ig-producing cells. Patients with common variable immunodeficiency have marked reduction in serum levels of both immunoglobulin G (IgG) and immunoglobulin A (IgA); about half of these patients also have reduced immunoglobulin M (IgM).', 'A primary immunodeficiency characterized by low levels or absence of all the immunoglobulin classes and lack of B-lymphocytes or plasma cells. It results in recurrent bacterial infections. Complications include autoimmune phenomena and cancer development.', 'Heterogeneous group of immunodeficiency syndromes characterized by hypogammaglobulinemia of most isotypes, variable B-cell defects, and the presence of recurrent bacterial infections.']

Data source
FinnGen phenocode D3_CVIDNAS
Hospital Discharge registry ICD-10: D838/D839
Cause of Death registry ICD-10: D839/D838
Level in the ICD-hierarchy 4
First defined in version DF2
Ontology
DOID 12177
GWAS catalog 0000367

Key figures

Sex All Female Male
Number of individuals 20 16 N/A
Unadjusted prevalence (%) 0.02 0.02 N/A
Mean age at first event (years) 46.87 48.29 N/A
Median number of events / individual 2 4 N/A

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 65.0 68.75 N/A
Case fatality at 5-years (%) 10.0 12.5 N/A

Associations