The remaining categories of PIRD which are not recognized as Tregopathies include hyperinflammatory disorders, such as hemophagocytic lymphohistocytosis (HLH) with or without intrinsic susceptibility to Epstein-Barr virus, non-malignant lymphoproliferation with autoimmunity (ALPS), inflammatory bowel disease linked to IL-10 signaling (very early onset inflammatory bowel disease, VEO-IBD), and other disorders linked to monogenic autoimmunity but lacking a central role in Treg (51, 53). Here, IL10 is linked to autoimmune lymphoproliferative syndrome.