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absence or severe deficiency of the plasma protein gamma globulin. There are three main types: transient, congenital, and acquired. The transient type occurs in early infancy, because gamma globulins are not produced in the fetus and the gamma globulins derived from the maternal blood are soon depleted. This temporary deficiency of gamma globulin lasts for the first 6 to 8 weeks, until the infant begins to synthesize the protein. Congenital agammaglobulinemia is a rare condition, occurring in males, and resulting in decreased or absent production of antibodies. Acquired agammaglobulinemia is secondary to other disorders and is usually a hypogammaglobulinemia, that is, a deficiency rather than total absence of this plasma protein. It is often secondary to malignant diseases such as leukemia, myeloma, and lymphoma, and to diseases associated with hypoproteinemia such as nephrosis and liver disease. Some patients have a family history of rheumatoid arthritis or allergies. This seems to indicate the presence of genetic factors in the development of agammaglobulinemia.
Symptoms. Because gamma globulin is so important in the production of antibodies and thus in the body's ability to defend itself against infection, it follows that a deficiency or absence of gamma globulin would result in severe and recurrent infections. The infections are usually bacterial rather than viral in origin and are extremely difficult to eliminate. The condition is often complicated by 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.
Treatment. Replacement therapy with human gamma globulin is effective in preventing severe infections. The aim is to maintain the gamma globulin level above 150 mg per 100 mL of blood. The optimal dose is determined by the patient's response. Antibiotics are also given and are continued until all signs of infection have disappeared. The prevention and management of infections requires close collaboration between all members of the health care team. The administration of live vaccines is contraindicated.
common variable agammaglobulinemia common variable immunodeficiency.
X-linked agammaglobulinemia a primary X-linked immunodeficiency disorder characterized by absence of circulating B lymphocytes, plasma cells, or germinal centers in lymphoid tissues, very low levels of circulating immunoglobulins, susceptibility to bacterial infection, and symptoms resembling rheumatoid arthritis. Pre-B cells apparently fail to differentiate into mature B cells, express surface immunoglobulins, and produce antibody.
Absence of, or extremely low levels of, the gamma fraction of serum globulin; sometimes used loosely to denote absence of immunoglobulins in general.
See also: hypogammaglobulinemia.
See also: hypogammaglobulinemia.
A congenital or acquired deficiency of gamma globulins in the blood.
AgammaglobulinemiaA rare condition characterised by an absence of antibodies due to an inability to produce immunoglobulins, which may be acquired or inherited as a genetic disease.
(1) X-linked agammaglobulinemia [MIM 300300], Bruton's disease.
(2) Autosomal recessive agammaglobulinemia [MIM 202500].
(3) X-linked severe combined immundeficiency syndrome [MIM 300400].
1. Swiss agammaglobulinemia, see there.
2. X-linked agammaglobulinemia, see there.
The lack of gamma globulins in the blood. Antibodies are the main gamma globulins of interest, so this term means a lack of antibodies.
Mentioned in: Immunodeficiency