Autoimmune Lymphoproliferative Syndrome

**Definition:** Rare congenital lymphoid disorder due to mutations in certain Fas-Fas ligand pathway genes. Known causes include mutations in FAS, TNFSF6, NRAS, CASP8, and CASP10 proteins. Clinical features include LYMPHADENOPATHY; SPLENOMEGALY; and AUTOIMMUNITY.

**Tree numbers:** - C15.604.515.138 - C16.320.089 - C20.111.288 - C20.683.515.124

**Synonyms:** - Canale Smith Syndrome - Canale-Smith Syndrome - Autoimmune Lymphoproliferative Syndrome, Type I, Autosomal Dominant