Ataxia-telangiectasia (A-T) is a rare, genetic disorder that affects the immune system, nervous system, and other body systems. People with A-T have an increased risk of developing certain types of cancer, as well as respiratory infections, neurologic disorders, and other health problems. The most common immunoglobulin absent in A-T is IgA (Immunoglobulin A).
What is Immunoglobulin A?
Immunoglobulin A (IgA) is an antibody that is found in the body to protect against infection and disease. It is the most abundant antibody in the body, and it is found in saliva, tears, and other secretions. IgA helps to protect the body from bacteria, viruses, and other foreign substances.
What Causes IgA Deficiency in Ataxia-Telangiectasia?
Ataxia-telangiectasia is caused by mutations in the ATM gene, which is responsible for producing a protein that helps to regulate cell division. Mutations in this gene can cause IgA levels to be lower than normal, leading to an IgA deficiency. This deficiency can increase the risk of infections, as well as other health problems.
How is an IgA Deficiency Treated?
The treatment for an IgA deficiency in A-T depends on the individual's symptoms and the severity of the deficiency. In some cases, doctors may recommend immunoglobulin therapy, which involves giving the patient an injection of a purified form of IgA. This can help to boost the body's ability to fight infection and may reduce the risk of complications due to an IgA deficiency. Other treatments may include antibiotics, antiviral medications, and other medications to help manage symptoms.