Key stages of bone marrow B-cell maturation are defective in patients with common variable immunodeficiency disorders

Common variable immunodeficiency disorders (CVIDs) comprise a heterogeneous group characterized by antibody production failure. Patients with CVID can have disease-related complications, including enteropathy, polyclonal lymphocytic infiltration, and/or autoimmune cytopenias, all of which are associated with increased lethality.1 In contrast, 40% to 60% of patients have no disease-related complications. In addition, infection-related structural damage, such as bronchiectasis, is found in around 50%.