X-Linked Agammaglobulinemia (XLA), sometimes called Bruton’s Agammaglobulinemia or Congenital Agammaglobulinemia, is inherited and means people lack the ability to produce antibodies, proteins that make up the gamma globulin, or immunoglobulin fraction of blood plasma.
Antibodies are an integral part of the body’s defense mechanism against certain microorganisms (e.g. bacteria, viruses). Antibodies are important in the recovery from infections and also protect against getting certain infections more than once.
People with XLA are prone to develop infections because they lack antibodies. The infections frequently occur in the middle ear, sinuses and lungs, but in some instances can also involve the bloodstream or internal organs. As a result, people with XLA may have infections that involve the sinuses (sinusitis), the eyes (conjunctivitis), the ears (otitis), the nose (rhinitis), the airways to the lung (bronchitis) or the lung itself (pneumonia). Gastrointestinal infections can also be a problem and some also have problems with skin infections. The infections may invade the bloodstream and spread to other organs deep within the body, such as the bones, joints or brain.
There is no cure patients who for XLA but people can be given some of the antibodies that they are lacking. The antibodies are supplied in the form of immunoglobulins (or gamma globulins) and can be given directly into the blood stream (intravenously) or under the skin (subcutaneously). Immunoglobulin is particularly effective in preventing the spread of infections into the bloodstream and to deep body tissues or organs. Some patients benefit from the use of oral antibiotics every day to protect them from infection or to treat chronic sinusitis or chronic bronchitis.
Patients with XLA should not receive any live viral vaccines, such as live polio, or the measles, mumps, rubella (MMR) vaccine.
Most X-Linked Agammaglobulinemia (XLA) patients who receive immunoglobulin on a regular basis will be able to lead relatively normal lives.
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