Primary agammaglobulinemia is an immune disorder related to antibody deficiency (hypogammaglobulinemia or low gamma
globulins) and is manifested in a variety of immune deficiency disorders in which the immune system is compromised.
This particular group of immune deficiencies may be the consequence of an inherited condition, an impaired immune system (from known or unknown causes), autoimmune diseases, or a malignancy.
Immunoglobulin deficiencies may be referred to by many different names, as there are several variables within the separate but related immune disorders; and there are also many subgroups. Antibody deficiency, immunoglobulin deficiency, and gamma globulin deficiency are all synonyms for hypogammaglobulinemia.
The goal of treatment is to reduce the number and severity of infections. Intramuscular injections of gamma globulins help to boost the immune system. Intravenous gamma globulin (including IgG, IgM, and IgA) gives a quick supplement to the immune system. High doses of high titer gamma globulin are needed for severe infections.
Antibiotics are useful for the treatment of bacterial infections. Treatment with immune globulins is vital to the health of people with agammaglobulinemia. Without treatment, most severe infections are fatal.