Common variable immunodeficiency:: the immune system in chaos

被引:62
作者
Bayry, J
Hermine, O
Webster, DA
Lévy, Y
Kaveri, SV
机构
[1] INSERM, U681, F-75006 Paris, France
[2] Univ Paris 06, Inst Cordeliers, F-75006 Paris, France
[3] Edward Jenner Inst Vaccine Res, Newbury RG20 7NN, Berks, England
[4] Hop Necker Enfants Malad, Dept Hematol, F-75015 Paris, France
[5] Hop Necker Enfants Malad, CNRS, UMR 8603, F-75015 Paris, France
[6] UCL, Royal Free Med Sch, Dept Immunol, Sch Med, London NW3 2QG, England
[7] Hop Henri Mondor, Serv Immunol Clin, F-94010 Creteil, France
关键词
D O I
10.1016/j.molmed.2005.06.005
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Common variable immunodeficiency (CVID) is a heterogeneous disorder that is associated with low serum-immunoglobulin concentrations, defective specific-antibody production and an increased susceptibility to bacterial infections of the respiratory and gastrointestinal tracts. In spite of the identification of genes that are associated with several known primary immunodeficiencies, the basic immunologic and molecular defects of the majority of patients with CAD have remained obscure. Most of the studies aimed at understanding the immunopathogenesis of CVID suggest that this condition is primarily a T-cell disorder, although renewed attention on the genetic linkage and haplotype analysis in families of patients with CVID and on the role of dendritic cells and B cells has revealed several interesting features. This new information should assist in understanding the pathogenesis of CVID and improving the therapeutic strategies.
引用
收藏
页码:370 / 376
页数:7
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