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

Publication date: Available online 28 November 2018

Source: Clinical Immunology

Author(s): Vassilios Lougaris, Daniele Moratto, Manuela Baronio, Tiziana Lorenzini, Stefano Rossi, Luisa Gazzurelli, Maria Pia Bondioni, Alessandro Plebani

Abstract

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.

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