Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6087259 | Clinical Immunology | 2016 | 4 Pages |
â¢CVID is an heterogeneous and complex disorder.â¢30% of evaluated CVID patients showed BM B cell developmental impairment.â¢CVID patients present peripheral B and T cell alterations, regardless of BM phenotype.â¢Combining these data with clinical features offers an alternative subdivision for CVID.
B cell developmental defects in CVID were recently described in a limited number of cases. To date, a detailed correlation between this maturational defect and the clinical presentation of affected patients has not been reported. In this study, we correlated bone marrow B cell evaluation, peripheral B and T lymphocyte subsets and clinical findings in 15 CVID patients. Early B cell developmental defects were observed in one third of patients. Combined bone marrow and peripheral lymphocytes evaluation allowed to further subdivide CVID patients in three groups with shared clinical features at diagnosis and during follow-up. These data broaden the number of CVID patients with early B cell developmental defects and, together with the peripheral lymphocytes evaluation, offer insight into the related clinical features in affected patients.