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Jerry A Winkelstein, MD
Section Editor
E Richard Stiehm, MD
Deputy Editor
Elizabeth TePas, MD, MS


Immunoglobulins are produced by plasma cells, which themselves are the result of the development and differentiation of B cells. Any factor that impedes the development of the B cell lineage and/or the function of mature B cells may result in levels of serum immunoglobulins that are reduced (ie, hypogammaglobulinemia) or nearly absent (ie, agammaglobulinemia). Primary agammaglobulinemia is most commonly inherited as an X-linked trait, but autosomal-recessive (AR) forms have also been reported. Only those inherited defects that are intrinsic to and limited to cells of the B cell lineage will be considered in this topic.

The following related issues are discussed separately:

Other primary immunodeficiency diseases that have agammaglobulinemia or hypogammaglobulinemia associated with combined B and T cell defects, and therefore are part of a broader immunodeficiency (see "Severe combined immunodeficiency (SCID): An overview" and "T-B-NK+ SCID: Pathogenesis and genetics" and "Syndromic immunodeficiencies").

Hypogammaglobulinemia that is secondary to extrinsic factors, such as malignancy, medications, and excessive loss of immunoglobulin (see "Secondary immunodeficiency due to underlying disease states, environmental exposures, and miscellaneous causes").

The various causes of hypogammaglobulinemia (see "Primary humoral immunodeficiencies: An overview" and "Transient hypogammaglobulinemia of infancy" and "IgG subclass deficiency" and "Specific antibody deficiency" and "IgG subclasses: Physical properties, genetics, and biologic functions" and "Pathogenesis of common variable immunodeficiency").


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Literature review current through: Sep 2016. | This topic last updated: Jul 30, 2014.
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