X-linked Agammaglobulinemia-Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs that work together to defend against germs. It helps your body to recognize these “foreign” invaders. Then its job is to keep them out, or if it can’t, to find and destroy them.

If your immune system cannot do its job, the results can be serious. Disorders of the immune system include

• Allergy and asthma – immune responses to substances that are usually not harmful
• Immune deficiency diseases – disorders in which the immune system is missing one or more of its parts
• Autoimmune diseases – diseases causing your immune system to attack your own body’s cells and tissues by mistake

NIH: National Institute of Allergy and Infectious Diseases

• Agammaglobulinemia (Medical Encyclopedia)
• Aging changes in immunity (Medical Encyclopedia)
• Chronic granulomatous disease (Medical Encyclopedia)
• Graft-versus-host disease (Medical Encyclopedia)
• Histiocytosis (Medical Encyclopedia)
• Hyperimmunoglobulin E syndrome (Medical Encyclopedia)
• Immune response (Medical Encyclopedia)
• Immunodeficiency disorders (Medical Encyclopedia)
• Selective deficiency of IgA (Medical Encyclopedia)

X-linked agammaglobulinemia (XLA) is a condition that affects the immune system and occurs almost exclusively in males. People with XLA have very few B cells, which are specialized white blood cells that help protect the body against infection. B cells can mature into the cells that produce special proteins called antibodies or immunoglobulins. Antibodies attach to specific foreign particles and germs, marking them for destruction. Individuals with XLA are more susceptible to infections because their body makes very few antibodies.Children with XLA are usually healthy for the first 1 or 2 months of life because they are protected by antibodies acquired before birth from their mother. After this time, the maternal antibodies are cleared from the body, and the affected child begins to develop recurrent infections. In children with XLA, infections generally take longer to get better and then they come back again, even with antibiotic medications. The most common bacterial infections that occur in people with XLA are lung infections (pneumonia and bronchitis), ear infections (otitis), pink eye (conjunctivitis), and sinus infections (sinusitis). Infections that cause chronic diarrhea are also common. Recurrent infections can lead to organ damage. People with XLA can develop severe, life-threatening bacterial infections; however, affected individuals are not particularly vulnerable to infections caused by viruses. With treatment to replace antibodies, infections can usually be prevented, improving the quality of life for people with XLA.