Dysgammaglobulinemia is a type of
immune disorder
An immune disorder is a dysfunction of the immune system. These disorders can be characterized in several different ways:
* By the component(s) of the immune system affected
* By whether the immune system is overactive or underactive
* By whether t ...
characterized by a reduction in some types of
gamma globulins, resulting in heightened susceptibility to some infectious diseases where primary immunity is antibody based.
It is distinguished from
hypogammaglobulinemia
Hypogammaglobulinemia is a problem with the immune system in which not enough gamma globulins are produced in the blood (thus '' hypo-'' + ''gamma'' + ''globulin'' + '' -emia''). This results in a lower antibody count, which impairs the immune sys ...
, which is a reduction in ''all'' types of gamma globulins.
Hyper IgM syndrome
Hyper IgM syndrome describes a group of primary immune deficiency disorders characterized by defective CD40 signaling; ''via'' B cells affecting class switch recombination (CSR) and somatic hypermutation. Immunoglobulin (Ig) class switch reco ...
can be considered a form of dysgammaglobulinemia, because it results from a failure of transformation from
IgM
Immunoglobulin M (IgM) is one of several isotypes of antibody (also known as immunoglobulin) that are produced by vertebrates. IgM is the largest antibody, and it is the first antibody to appear in the response to initial exposure to an antig ...
production to production of other antibodies, and so the condition can be interpreted as a reduction of the other types.
See also
*
Immunodeficiency
References
Further reading
* Raif S. Geha et al.:
Hyper Immunoglobulin M Immunodeficiency (Dysgammaglobulinemia), ''Journal of Clinical Investigation'', 1979 August; 64(2): 385–391, . Accessed 2009-07-17.
* Andre Cruchaud et al.:
The site of synthesis of the 19S T-globulins in dysgammaglobulinemia (1962). Accessed 2009-07-17.
External links
Predominantly antibody deficiencies
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