Cumulative frequency of patients having developed lymphoproliferation and autoimmunity as a function of age. (A) Splenomegaly precedes the development of lymphadenopathy and autoimmune manifestations. (B) Lymphodemopathy develops in 72% of patients. (C) Autoimmunity occured in 75% of patients. ++ indicates lymphadenopathy > 2 cm and < 5 cm; +++, lymphadenopathy > 5 cm; and ADP, lymphadenopathy.