Primary B-cell immunodeficiencies

Primary B-cell immunodeficiencies refer to diseases resulting from impaired antibody production due to either molecular defects intrinsic to B-cells or a failure of interaction between B-cells and T-cells. Patients typically have recurrent infections and can vary with presentation and complications...

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Bibliographic Details
Published in:Human immunology 2019-06, Vol.80 (6), p.351-362
Main Authors: Smith, Tukisa, Cunningham-Rundles, Charlotte
Format: Article
Language:English
Subjects:
Agammaglobulinemia
Antibody
B-cell receptor
Common variable immunodeficiency
Hyper IgM syndrome
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Summary:Primary B-cell immunodeficiencies refer to diseases resulting from impaired antibody production due to either molecular defects intrinsic to B-cells or a failure of interaction between B-cells and T-cells. Patients typically have recurrent infections and can vary with presentation and complications depending upon where the defect has occurred in B-cell development or the degree of functional impairment. In this review, we describe B-cell specific immune defects categorized by presence or absence of peripheral B-cells, immunoglobulins isotypes and evidence of antibody impairment.
ISSN:0198-8859
1879-1166
DOI:10.1016/j.humimm.2018.10.015