Autoimmune lymphoproliferative syndrome (ALPS): A disease caused by failure of lymphocytes to die once they have finished doing their job. As a result, lymphocytes hang around in the spleen and lymph nodes which grow large, and immune cells attack the body's own tissues, a condition known as autoimmunity.

ALPS is characterized by:

• Anemia due to excessive destruction of red blood cells hemolytic anemia ),
• Decreased platelets in the blood (thrombocytopenia),
• Enlargement of lymph nodes ( lymphadenopathy ), and
• Enlargement of the spleen (splenomegaly).

On a genetic level there are two types of ALPS. Type I ALPS ("classic" ALPS) is caused by mutations in a cell surface protein called FAS or in its ligand FASL which play a pivotal role in the programmed cell death (a process known as apoptosis ) of lymphocytes. Defects in the expression of FAS or FASL result in the abnormal accumulation of mature lymphocytes and in autoimmune disease. Type II ALPS is caused by mutation in caspase-10, a gene involved in the cascade of protease reactions responsible for programmed cell death (apoptosis).

ALPS was first reported in 1967 by Canale and Smith. It is sometimes called the Canale-Smith syndrome.

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