agammaglobulinemia /agam′əglob′yo̅o̅linē″mē·ə/ [Gk, a + gamma, not gamma (third letter of Greek alphabet); L, globulus, small sphere; Gk, haima, blood] , the absence of gamma globulin from the serum, associated with an increased susceptibility to infection. The condition may be transient, congenital, or acquired. The transient form is common in infancy before 6 weeks of age, when the infant becomes able to synthesize the immunoglobulin. The congenital form is rare and sex-linked, affecting male children; it results in decreased production of antibodies. The acquired form usually occurs in malignant diseases such as leukemia, myeloma, or lymphoma. Also spelled agammaglobulinaemia. See also Bruton’s agammaglobulinemia, immune gamma globulin. ▪ OBSERVATIONS: Symptoms are related to a wide variety of bacterial infections, including but not limited to bronchitis, diarrhea, conjunctivitis, and pneumonia. Blood tests indicating low levels of immunoglobulins confirm the diagnosis. ▪ INTERVENTIONS: Replacement therapy with human gamma globulin is effective in preventing severe infections. The aim is to maintain the gamma globulin level above 150 mg/100 mL of blood. Antibiotics are administered and are continued until all signs of infection are eliminated. ▪ PATIENT CARE CONSIDERATIONS: Recurrent infections are not uncommon. The condition is also complicated by local damage to tissues related to scarring from the frequent infections. Genetic counseling should be considered for prospective parents with a family history of the disorder.
