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Pathol Biol (Paris) ; 62(6): 377-81, 2014 Dec.
Article in French | MEDLINE | ID: mdl-25200463

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 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.


Subject(s)
Common Variable Immunodeficiency/immunology , Common Variable Immunodeficiency/pathology , Immunophenotyping , Adolescent , Adult , Algeria/epidemiology , Child , Child, Preschool , Common Variable Immunodeficiency/epidemiology , Female , Humans , Infant , Male , Middle Aged , Retrospective Studies , Young Adult
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