Hypogammaglobulinaemia Health Dictionary

Impaired IMMUNITY resulting from inherited or acquired abnormalities of the immune system. This leads to increased vulnerability to infection. Important inherited examples of immunode?ciency are defects in function of GRANULOCYTES and the COMPLEMENT SYSTEM. Common acquired forms of immunode?ciency are defective function of B-type lymphocytes and hence antibody de?ciency in ‘common variable hypogammaglobulinaemia’, and grossly de?cient CD4 T-cell function – malfunctioning T-type lymphocytes – in AIDS, secondary to HIV infection (see AIDS/HIV).... immunodeficiency

Disorders in which there is a failure of the immune system’s defences to fight infection and tumours. They may be due to an inherited or a congenital defect or may be the result of acquired disease. The result is persistent or recurrent infection, including those with organisms that would not ordinarily cause disease, and an undue susceptibility to certain forms of cancer. The infections in people with immunodeficiency disorders are sometimes called opportunistic infections; examples include pneumocystic pneumonia, fungal infections, and widespread herpes simplex infections.

Congenital or inherited deficiencies can occur in either of the 2 prongs of the adaptive immune system: humoral or cellular. Deficiencies of the humoral system include hypogammaglobulinaemia and agammaglobulinaemia. The former may cause few or no symptoms, depending on the severity of the deficiency, but agammaglobulinaemia can be fatal if not treated with immunoglobulin. Congenital deficiencies of T-lymphocytes may lead to problems such as persistent and widespread candidiasis (thrush). A combined deficiency of both humoral and cellular components of the immune system, called severe combined immunodeficiency (SCID), is usually fatal in the 1st year of life unless treatment can be given by bone marrow transplant.

Acquired immunodeficiency may be due either to disease processes (such as infection with HIV, which leads to AIDS) or damage to the immune system as a result of its suppression by drugs. Severe malnutrition and many cancers can also cause immunodeficiency. Mild immunodeficiency arises through a natural decline in immune defences with age.... immunodeficiency disorders

n. a total deficiency of the plasma protein *gammaglobulin. Compare hypogammaglobulinaemia.... agammaglobulinaemia

n. a decrease in the quantity of protein in the blood. It may result from malnutrition, impaired protein production (as in liver disease), or increased loss of protein from the body (as in the *nephrotic syndrome). It results in swelling (*oedema), because of the accumulation of fluid in the tissues, and increased susceptibility to infections. See also hypogammaglobulinaemia.... hypoproteinaemia

n. deficiency in the *immune response. This can be acquired, as in *AIDS, but there are many varieties of primary immunodeficiency occurring as inherited disorders characterized by *hypogammaglobulinaemia or defects in T-cell function, or both.... immunodeficiency

any one of a group of inherited conditions in which there is a disturbance in either the structure, synthesis, function, or transport of protein molecules. There are over 1500 inborn errors of metabolism; examples are *phenylketonuria, *homocystinuria, and *hypogammaglobulinaemia.... inborn error of metabolism