Autoimmune Lyphoproliferative Syndrome (ALPS) (sometimes referred to as Canale-Smith Syndrome) is a disorder when the body makes an abnormally large amount of lymphocytes (immune system cells), causing enlarged lymph nodes, liver, and spleen. Autoimmune Lyphoproliferative Syndrome is an inherited disease in an autosomal dominant pattern. The autoimmune component is present with the immune system attacking the cells within the blood stream. Mutations in the FASgene account for 75% of ALPS cases as this gene codes for proteins that regulate cell apoptosis. When this mutation is present, the lymphocytes do not participate in apoptosis because they have not been told by the protein to, resulting in an excess number of lymphocytes in the body. Autoimmune Lyphoproliferative Syndrome is typically diagnosed in children. Treatments include a splenectomy, steroids, and blood transfusions to treat anemia.
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Autoimmune Lymphoproliferative Syndrome is also known as autoimmune lymphoproliferative syndrome, canale-smith syndrome, autoimmune lymphoproliferative syndrome type 1, autosomal dominant, alps (autoimmune lymphoproliferative syndrome), fas deficiency.