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Published on: July 11, 2011
Primary B-cell immunodeficiencies
Tukisa Smith1, Charlotte Cunningham-Rundles2
1Division of Allergy and Clinical Immunology, Icahn School of Medicine at Mount Sinai, 1425 Madison Avenue, New York, NY 10029-6574, United States; The Rockefeller University, Laboratory of Biochemical Genetics and Metabolism, 1230 York Avenue, Box 179, New York, NY 10065, United States.
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.
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.
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