Agammaglobulinemia: Overview, Causes

Alternate Names : Bruton’s agammaglobulinemia, X-linked agammaglobulinemia

Definition

Agammaglobulinemia is an inherited disorder in which there are very low levels of protective immune system proteins called immunoglobulins. People with this disorder repeatedly develop infections.

Overview, Causes, & Risk Factors

Agammaglobulinemia is a rare disorder that mainly affects males. It is the result of a genetic abnormality that blocks the development of normal, mature immune system cells called B lymphocytes.

As a result, the body produces very little (if any) immunoglobulins in the bloodstream. Immunoglobulins play a major role in the immune response, which protects against illness and infection.

Without protective immunoglobulins, people with agammaglobulinemia repeatedly develop infections. People with this disorder are particularly susceptible to bacterial infections caused by Haemophilus influenzae, pneumococci (Streptococcus pneumoniae), and staphylococci, as well as to repeated viral infections. Common sites of infection include:

* Gastrointestinal tract
* Lungs
* Skin
* Upper respiratory tract

People with this condition may have a family history of agammaglobulinemia (or another immune disorder).

Pictures & Images

Agammaglobulinemia

Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

Agammaglobulinemia: Overview, Causes

Agammaglobulinemia: Symptoms & Signs, Diagnosis & Tests

Agammaglobulinemia: Treatment


Review Date : 5/16/2010
Reviewed By : David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Stuart I. Henochowicz, MD, FACP, Associate CLinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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