ADA2 and splenic infarction: Given the high proportion of patients (three out of 10 in our cohort and three out of seven previously reported in literature) with CNS vascular events and renal/splenic infarction (one out of seven previously reported in literature), our findings invite for a careful reconsideration of treating carriers of dominant negative ADA2 variants with TNFi, as per current guidelines for AR ADA2.