In this video, V. Koneti Rao, MD, FRCPA, National Institute of Allergy and Infectious Diseases, Bethesda, MD talks on the pathogenesis and treatment of autoimmune lymphoproliferative syndrome (ALPS). Dr Rao explains how ALPS is a disorder of apoptosis caused by a defect in the FAS gene, and then highlights the management of this disease with immunomodulatory treatment. The similarities and differences between the clinical presentation of patients with activated PI3K delta syndrome (APDS/PASLI) compared to patients with ALPS are discussed, as well as how both disorders increase the risk of developing Hodgkin or non-Hodgkin lymphoma (NHL). This interview took place at the European Hematology Association (EHA) Congress 2022 held in Vienna, Austria.