Immunologic Research

, Volume 38, Issue 1–3, pp 78–86

Common variable immune deficiency: reviews, continued puzzles, and a new registry

Article

Abstract

Background

Common variable immune deficiency (CVID) is a clinically and immunologically heterogenous primary immune deficiency first described more than 50 years ago. The main features are hypogammaglobulinemia, recurrent infections, and other complications. While CVID is considered as a genetic immune defect, and several genes have been reported as leading to the CVID phenotype, one of the most puzzling features of CVID is the sporadic inheritance pattern and the relatively late onset. In most cases, no other family members have any immune defect. The mean age at diagnosis is between 25 and 45 years of age. These features suggest the interplay between either several or numerous genes with or without potential environmental factors.

Keywords

Common variable immune deficiency B cells Genetics Autoimmunity Immune deficiency 

Abbreviations

CVID

Common variable immune deficiency

ICOS

Inducible costimulator

IDF

Immune Deficiency Foundation

ITP

Idiopathic thrombocytopenia purpura

TACI

Transmembrane activator and calcium-modulator and cyclophilin-ligand interactor

USID Net

United States Immune Deficiency Network

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Copyright information

© Humana Press Inc. 2007

Authors and Affiliations

  • Charlotte Cunningham-Rundles
    • 1
    • 2
    • 3
    • 4
  • Adina Kay Knight
    • 1
    • 2
    • 3
    • 5
  1. 1.Department of MedicineMount Sinai Medical Center, The Mount Sinai School of MedicineNew YorkUSA
  2. 2.Department of PediatricsMount Sinai Medical Center, The Mount Sinai School of MedicineNew YorkUSA
  3. 3.The Immunobiology CenterMount Sinai Medical Center, The Mount Sinai School of MedicineNew YorkUSA
  4. 4.New YorkUSA
  5. 5.BirminghamUSA

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