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Alternative names Return to top
Bruton's agammaglobulinemia; X-linked agammaglobulinemiaDefinition Return to top
Agammaglobulinemia is an inherited disorder characterized by very low levels of protective immunoglobulins; affected people develop repeated infections.Causes, incidence, and risk factors Return to top
Agammaglobulinemia is rare disorder that primarily affects males, because it is inherited as an X-linked recessive trait (like hemophilia). It is the result of a genetic abnormality that blocks the development of normal, mature B-lymphocyte cells. As a consequence, the body produces very little (if any) gamma globulin (IgG) and other immunoglobulins in the bloodstream. Immunoglobulins are molecules that play a significant role in the immune response, which protects against illness and infection.
In the absence of protective immunoglobulins, affected individuals develop repeated infections. People with this disorder are particularly susceptible to bacterial infections caused by Hemophilus influenzae, pneumococcus (Streptococcus pneumoniae), and staphylococci as well as repeated viral infections. The upper respiratory tract, lungs, and skin are common sites of infection.
Symptoms Return to top
Signs and tests Return to top
The disorder is confirmed by laboratory measurement of serum immunoglobulins. Tests include:Treatment Return to top
The goal of treatment is to reduce the number and severity of infections as well as provide genetic counseling to affected families.Expectations (prognosis) Return to top
Treatment with immune globulins has improved the health of people with agammaglobulinemia. Without treatment, most severe infections are fatal.Complications Return to top
Calling your health care provider Return to top
Call for an appointment with your health care provider if you or your child has experienced frequent infections.Prevention Return to top
Genetic counseling may be offered to prospective parents with a family history of agammaglobulinemia or other immunodeficiency disorders. Update Date: 5/24/2004 Updated by: A.D.A.M. editorial. Previously reviewed by Donald Accetta, MD, MPH. President, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network (10/30/2003).
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Page last updated: 28 October 2004 |