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agammaglobulinemia
(a-gam″ә-glob″u-lĭ-ne´me-ә)
absence or severe deficiency of the plasma protein gamma globulin. This is seen transiently in babies immediately after birth, because gamma globulins are not produced in the fetus and those
derived from the mother's blood are soon depleted. After 6 to 8 weeks, the infant should begin to synthesize gamma globulin
on its own. A congenital form, X-linked agammaglobulinemia, is a rare condition seen in males. Acquired types of agammaglobulinemia are secondary to other disorders and usually severe
forms of hypogammaglobulinemia. Because gamma globulin is so important in the production of antibodies and the body's ability to defend itself against infection, a deficiency or absence of gamma globulin results in severe and
recurrent infections, usually bacterial ones that are difficult to eliminate. There is often local damage to tissues because
of scarring and repeated infection. Disorders of connective tissue such as scleroderma, arthritis, and lupus erythematosus are also frequent complications.
common variable agammaglobulinemia
common variable immunodeficiency.
X-linked agammaglobulinemia
a primary X-linked immunodeficiency disorder in which boys lack circulating B lymphocytes and plasma cells, do not have germinal centers in the lymphoid tissue,
and have low levels of circulating immunoglobulins; they are susceptible to bacterial infection and often have symptoms similar
to those of rheumatoid arthritis.
Copyright 2007. An Elsevier publication. All rights reserved.
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