Canale-Smith syndrome | definition of Canale-Smith syndrome by Medical dictionary
autoimmune lymphoproliferative syndrome A condition caused by defective lymphocyte homeostasis and characterised by:
• Non-malignant lymphoproliferation (lymphadenopathy, hepatosplenomegaly with or without hypersplenism) that often improves with age;
• Autoimmune disease, mostly directed toward peripheral blood cells; and
• Lifelong increased risk of Hodgkin and non-Hodgkin lymphoma.
Canale-Smith syndrome An AUTOIMMUNE disease caused by mutations of the Fas gene, or defects in the Fas expression cascade, that leads to defective APOPTOSIS in lymphocytes. The result is a lymphoproliferative disorder with lymphadenopathy, spleen enlargement, possible lymphoma development and other effects including excess gamma globulin production.