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.

中文翻译：

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在无自身免疫的情况下，NFKB2突变的CVID疾病中的早期B细胞发育损伤伴进行性B细胞缺乏。

这项研究为NFKB2在骨髓和周围人B细胞发育中的新作用提供了证据，从而导致进行性外周B细胞缺乏症并不总是与自身免疫现象结合，从而拓宽了NFKB2突变的CVID疾病的临床范围，并暗示NFKB2在早期人类B细胞发育中的重要作用。