NFKB1 and common variable immunodeficiency: Four of the 32 patients had a possible monogenic defect with a known association to CVID, two of these patients were in the progressive group [CTLA4-haploinsufficieny not previously described variant but likely pathogenic; STAT3 variant of uncertain significance (VUS)] and two in the stable group (NFkB1 and BACH2, both VUS).