Section 16. Immune Disorders

Chapter 184. Immunodeficiency Disorders

Topics: Introduction | X-Linked Agammaglobulinemia | Selective Antibody Deficiency | Common Variable Immunodeficiency | Transient Hypogammaglobulinemia of Infancy | Chronic Mucocutaneous Candidiasis | DiGeorge Anomaly | Ataxia-Telangiectasia | Severe Combined Immunodeficiency Disease | Wiskott-Aldrich Syndrome | Hyperimmunoglobulinemia E Syndrome | Chronic Granulomatous Disease | Immunodeficiency due to Spleen Disorders

X-Linked Agammaglobulinemia

X-Linked agammaglobulinemia (Bruton's agammaglobulinemia) is a hereditary immunodeficiency disorder due to an abnormality in the X chromosome and resulting in few or no B lymphocytes and very low levels of antibodies.

X-Linked agammaglobulinemia affects only boys. For about the first 6 months after birth, antibodies from the mother protect against infection. At about age 6 months, affected infants start having recurring infections of the ears, sinuses, lungs, and bones, usually due to bacteria such as pneumococcus, haemophilus, and streptococcus. Some unusual viral infections of the brain may develop. The risk of cancer is increased.

Infusions of immune globulin are given throughout life to help prevent infections. Antibiotics are promptly given to treat bacterial infections and may be given continuously. Despite these measures, chronic sinus and lung infections often develop. With treatment, lifespan may be unaffected.