Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease defined as a defect in the lymphocyte apoptotic pathway and characterized by chronic massive, nonmalignant lymphadenopathy, splenomegaly and/or hepatomegaly; expansion of a normally rare population of T cells bearing ab-antigen receptors but lacking both CD4 and CD8 coreceptors (α/β double-negative T cells, α/βDNTs) and defective in vitro apoptosis of mature lymphocytes [3,4]. Here, CD4 is linked to autoimmune lymphoproliferative syndrome.