Other variants in NFKB1 in CVID have been described as the most common cause of CVID with non-infectious complications, including lymphadenopathy, splenomegaly, and autoimmune disease, and all reported patients exhibit deficient B-lymphocyte differentiation with increased CD21low B-cell numbers (21, 32). Here, NFKB1 is linked to common variable immunodeficiency.