Déficit immunitaire commun variable (DICV) : caractéristiques cliniques et immunologiques de 29 patients algériens

Abstract

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 29 patients fulfilling the classical CVID definition. Blood tests included immunoglobulin measurement and lymphocyte subpopulations phenotyping.

Results

This study includes 29 patients. 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.