Thymic hypoplasia is a disorder where the thymus gland is underdeveloped, making people more prone to infections. It can occur as part of DiGeorge syndrome, which can cause heart defects, facial abnormalities, seizures, and learning disabilities. Treatment may involve a bone marrow or thymus tissue transplant, or immunoglobulin therapy. The outlook depends on the degree of thymic function loss and other associated conditions.
Thymic hypoplasia is a disorder in which there is underdevelopment of the thymus gland. The thymus is located under the breastbone, or breastbone, and is part of the immune system. Thymic hypoplasia can make people more prone to infections and can occur as part of a congenital condition, present from birth, known as DiGeorge syndrome. DiGeorge syndrome can be associated with problems such as heart defects, facial abnormalities, seizures, and learning disabilities. The degree of thymus hypoplasia can range from moderate to severe, and in some cases the thymus may be missing.
As part of the immune system, the thymus gland produces white blood cells known as T lymphocytes, which are involved in the body’s response to infection. In most people, the thymus only develops until adolescence. From then on, the amount of cell growth decreases and the thymus begins to shrink, with the active cells slowly being replaced by scar tissue and fat. Despite this shrinkage, the gland still produces lymphocytes and continues to do so into old age.
Fortunately, only a small percentage of people with DiGeorge syndrome have thymic aplasia, where there is no gland, or hypoplasia, which is severe enough to leave them prone to serious infections. For those with moderate thymic hypoplasia, recurring infections may be a problem, but they are more likely to be less serious illnesses such as a cold. Many children with moderate thymic hypoplasia may even find that their immune system function improves as they age.
Where gland function is severely impaired, treatment of thymic hypoplasia might involve a bone marrow transplant or a transplant of thymus tissue from a donor. Sometimes, immunoglobulin therapy may be done. This involves the administration of immunoglobulins, or antibodies, extracted from donated blood plasma. Antibodies are special proteins that help defend the body against infection, and this type of treatment is given into a vein.
The outlook for a person with thymic hypoplasia depends in part on the degree of loss of thymic function. It is also affected by the extent to which other organs, such as the heart, are defective, as part of DiGeorge syndrome. Behavior, mental health, and developmental issues can also occur as part of the condition and can make it more difficult to manage. A team of experts from a range of disciplines is usually needed to provide adequate care.
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