Early B cell developmental impairment with progressive B cell deficiency in NFKB2 mutated CVID disease without autoimmunity.

This study provides evidence for a novel role for NFKB2 in human B cell development in the bone marrow and in
the periphery, leading to progressive peripheral B cell deficiency not always combined with autoimmune phenomena,
broadening thus the clinical spectrum of NFKB2 mutated CVID disease and implying an essential role
for NFKB2 in early human B cell development.