In a group of CVID patients, plasma protein profiling identified a Th1-driven immune dysregulation, with increased plasma levels of IFN-γ and of proteins regulated by IFN-γ, NF-kB2 and NF-kB1 haploinsufficiency, or increased plasma levels of CXCL13 reflecting aberrant germinal center (GC) formation (12). Here, NFKB2 is linked to common variable immunodeficiency.