Clearly overwhelming post-splenectomy sepsis is a major cause of morbidity and mortality.119–121 Therefore nowadays, avoidance of splenectomy is recommended,119–120 so, the prognosis for ALPS-FAS is improving and depends, on steroid-sparing management of cytopenias with mycophenolate mofetil or sirolimus, and vigilance for lymphoma.120,121. Here, FAS is linked to autoimmune lymphoproliferative syndrome.