Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function

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.']

Endpoint definition

Name D3_CVID_BCELL
Long name Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
Hospital Discharge registry ICD-10: D830
Cause of Death registry ICD-10: D830
Level in the ICD-hierarchy 4
First defined in version DF2
Latin name Deficientia immunalis variabilis communis praecipue cum abnormalitatibus numeri et functionis cellularum B

Summary Statistics

Key figures

All Female Male
Number of individuals 40 25 15
Unadjusted prevalence (%) 0.02 0.02 0.02
Mean age at first event (years) 45.92 45.72 46.26
Case fatality at 5-years (%) 0.00 0.00 0.00

Longitudinal metrics

All Female Male
Median nb. of events per indiv. 3.0 2.0 5.0
Recurrence at 6 months (%) 57.50 48.00 73.33

Survival analyses between endpoints

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