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Immunophenotypic and Functional Analysis of Lymphocyte Subsets in Common Variable Immunodeficiency Patients Without Monogenic Defects Publisher Pubmed



Tofighi Zavareh F1, 2, 3 ; Mirshafiey A1, 2 ; Yazdani R1, 3, 4 ; Keshtkar AA5 ; Abolhassani H1, 6, 7 ; Mahdaviani SA8 ; Habibi S1 ; Sohani M1 ; Rezaei N1, 3, 9 ; Aghamohammadi A1
Authors
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Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  4. 4. Department of Neurology, Thomas Jefferson University, Philadelphia, PA, United States
  5. 5. Department of Health Sciences Education Development, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran
  6. 6. Division of Clinical Immunology, Department of Biosciences and Nutrition, Karolinska Institute, Stockholm, Sweden
  7. 7. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
  8. 8. Pediatric Respiratory Diseases Research Centre, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
  9. 9. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Scandinavian Journal of Immunology Published:2022


Abstract

Common variable immunodeficiency (CVID) is accompanied by various lymphocyte abnormalities believed to be mostly responsible for disease features in patients with no diagnosed monogenic defects. Here, we evaluated the association of B and T lymphocyte abnormalities with the incidence of CVID. Twenty-six genetically unsolved CVID patients were examined for B and T lymphocyte subsets by flow cytometry and CD4+ T-cell proliferation by carboxyfluorescein succinimidyl ester (CFSE) test. We detected a reduction in total, naive, memory B cells and plasmablasts, and also total, naive, central memory and regulatory CD4+ T cells, besides naive CD8+ T cells. There was an increase in CD21low and transitional B cells, effector memory (EM) and terminally differentiated effector memory (TEMRA) CD4+ T-cell subsets as well as total, EM, TEMRA, activated and cytotoxic CD8+ T cells among non-monogenic CVID patients. CD4+ T-cell proliferation response was reduced regarding both division index and percent divided. In conclusion, regarding the similarity of lymphocyte abnormalities between patients without genetic defects and those with monogenic defects, genetic mutations are not responsible for these specific lymphocyte changes. However, the novel correlations observed between lymphocyte alterations among genetically unsolved CVID patients may serve as a guide to predict the potential of future CVID development for hypogammaglobulinemia children. © 2022 The Scandinavian Foundation for Immunology.
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