Pathologie-biologie · 2014
[Common variable immunodeficiency (CVID): clinical and immunological features of 29 Algerian patients].
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Tahiat A, Djidjik R, Boushaki S, Cherguelaïne K, Gharnaout M, Boumedine S, Smati L, Benhalla N, Atek A, Baghriche M, Zidouni N, Ghaffor M
Аннотация
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 23years. 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.
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 23years. 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.