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Abnormalities of the thymus can result in a decreased number of T cells and autoimmune diseases such as autoimmune polyendocrine syndrome type 1 and myasthenia gravis. These are often associated with cancer of the tissue of the thymus, called thymoma, or tissues arising from immature lymphocytes such as T cells, called lymphoma.
AIRE is a transcription factor expressed in the medulla [broken anchor] (inner part) of the thymus. It is part of the mechanism which eliminates self-reactive T cells that would cause autoimmune disease. It exposes T cells to normal, healthy proteins from all parts of the body, and T cells that react to those proteins are destroyed.
A thymoma is a tumor originating from the epithelial cells of the thymus that is considered a rare neoplasm. [1] Thymomas are frequently associated with neuromuscular disorders such as myasthenia gravis; [2] thymoma is found in 20% of patients with myasthenia gravis. [3] Once diagnosed, thymomas may be removed surgically.
Thymoma is a common neoplasm arising from the thymus, the primary lymphoid organ where T cells become educated to distinguish "self" from "non self". In the setting of thymoma, abnormal thymic education occurs as a result of subtle differences in antigen processing. In TAMA these differences result in autoreactive T cells escaping from the thymus.
Thymic involution is the shrinking of the thymus with age, resulting in changes in the architecture of the thymus and a decrease in tissue mass. [1] Thymus involution is one of the major characteristics of vertebrate immunology, and occurs in almost all vertebrates, from birds, teleosts, amphibians to reptiles, though the thymi of a few species of sharks are known not to involute.
Autoimmune disease is a frequent complication after human allogeneic thymus transplantation, found in 42% of subjects over 1 year post transplantation. [9] However, this is partially explained by that the indication itself, that is, complete DiGeorge syndrome, increases the risk of autoimmune disease. [2]
Thymus hyperplasia refers to an enlargement ("hyperplasia") of the thymus. [1] It is not always a disease state. The size of the thymus usually peaks during adolescence and atrophies in the following decades. Before the immune function of the thymus was well understood, the enlargement was sometimes seen as a cause for alarm, and justification ...
Nezelof syndrome is an autosomal recessive [6] congenital immunodeficiency condition due to underdevelopment of the thymus. The defect is a type of purine nucleoside phosphorylase deficiency with inactive phosphorylase, this results in an accumulation of deoxy-GTP which inhibits ribonucleotide reductase .