Germline absence of CARD11 causes combined immunodeficiency (CID), whereas germline hypomorphic and GoF mutations drive the immune dysregulation syndromes CARD11-associated atopy with dominant interference of NF-κB signaling (CADINS) and B-cell expansion with NF-κB and T-cell anergy (BENTA), respectively (DeVore and Hershey, 2022). Here, NFKB1 is linked to severe combined immunodeficiency.