XIAP and Splenomegaly: The most commom characterization of XIAP deficiency is a key triad of clinical manifestations, including a high susceptibility to developing haemophagocytic lymphohistiocytosis (HLH) frequently triggered by Epstein–Barr virus (EBV) infection, recurrent splenomegaly and inflammatory bowel disease (IBD) with the features of Crohn’s disease.