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Common Variable Immune Deficiency: Reviews, Continued Puzzles, and a New Registry

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Journal Immunol Res
Date 2007 Oct 6
PMID 17917013
Citations 15
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Abstract

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.

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References
1.
Pollack S, Reisner Y, Koziner B, GOOD R, Hoffman M . B-cell function in common variable immunodeficiency: suppression of in vitro anti-sheep erythrocytes antibody production by T cells and monocytes. Immunology. 1985; 54(1):89-96. PMC: 1454853. View

2.
Cunningham-Rundles C . Clinical and immunologic analyses of 103 patients with common variable immunodeficiency. J Clin Immunol. 1989; 9(1):22-33. DOI: 10.1007/BF00917124. View

3.
Chun H, Zheng L, Ahmad M, Wang J, Speirs C, Siegel R . Pleiotropic defects in lymphocyte activation caused by caspase-8 mutations lead to human immunodeficiency. Nature. 2002; 419(6905):395-9. DOI: 10.1038/nature01063. View

4.
Bryant A, Calver N, Toubi E, Webster A, Farrant J . Classification of patients with common variable immunodeficiency by B cell secretion of IgM and IgG in response to anti-IgM and interleukin-2. Clin Immunol Immunopathol. 1990; 56(2):239-48. DOI: 10.1016/0090-1229(90)90145-g. View

5.
SANFORD J, FAVOUR C, TRIBEMAN M . Absence of serum gamma globulins in an adult. N Engl J Med. 1954; 250(24):1027-9. DOI: 10.1056/NEJM195406172502403. View