autoimmune lymphoproliferative syndrome

(redirected from FASLG-related autoimmune lymphoproliferative syndrome)

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.