Common variable immunodeficiency (CVID): Clinical and immunological features of 29Algerian patients

Purpose: Common variable immunodeficiency (CVID) is the commonest symptomatic primary immunodeficiency. It is characterized by a defect of antibody production, recurrent respiratory tract infections and increased occurrence of auto-immune discords and lymphoproliferative disease. Methods: This retrospective study was conducted on 29patients fulfilling the classical CVID definition. Blood tests included immunoglobulin measurement and lymphocyte subpopulations phenotyping. Results: This study includes 29patients. The mean age at diagnosis was 23. years. Recurrent upper and lower bacterial respiratory tract infections were common in almost all patients. Five patients developed auto-immune conditions and six had lymphoproliferative disease. Decreased IgG was found in almost all patients. Low IgA and IgM levels were found in 89.6% and 65.5% of cases respectively. Abnormal T and/or B phenotype was found in 75% of cases; the most common abnormalities were decreased circulating B (54.2%) and T CD4+ (41.7%) cells and inversion of the CD4/CD8 ratio (70.8%). Patients with decreased circulating B and T CD4+ cells were significantly more likely to have auto-immune cytopenias and lymphoproliferative disease. Conclusions: Our study confirms the heterogeneity of CVID. A patient's classification is necessary to define homogeneous groups of patients and to characterize specific molecular abnormalities in each group.