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B Cells and T Cells Abnormalities in Patients With Selective Iga Deficiency Publisher



Bagheri Y1, 2 ; Moeini Shad T3, 4 ; Namazi S5 ; Tofighi Zavareh F3, 6 ; Azizi G7 ; Salami F3, 4 ; Sadani S8 ; Hosseini A8 ; Saeidi M2, 9 ; Pashangzadeh S3 ; Delavari S3, 4 ; Mirminachi B10 ; Rezaei N3, 11, 12 ; Abolhassani H3, 13 Show All Authors
Authors
  1. Bagheri Y1, 2
  2. Moeini Shad T3, 4
  3. Namazi S5
  4. Tofighi Zavareh F3, 6
  5. Azizi G7
  6. Salami F3, 4
  7. Sadani S8
  8. Hosseini A8
  9. Saeidi M2, 9
  10. Pashangzadeh S3
  11. Delavari S3, 4
  12. Mirminachi B10
  13. Rezaei N3, 11, 12
  14. Abolhassani H3, 13
  15. Aghamohammadi A3
  16. Yazdani R3, 4, 13, 14
Show Affiliations
Authors Affiliations
  1. 1. Cancer Research Center, Golestan University of Medical Sciences, Gorgan, Iran
  2. 2. Department of Immunology, School of Medicine, Golestan University of Medical Sciences, Gorgan, Iran
  3. 3. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center Hospital, Tehran University of Medical Sciences, 62 Qarib St., Keshavarz Blvd., Tehran, 14194, Iran
  4. 4. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  5. 5. Department of Immunology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
  6. 6. Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  8. 8. Clinical Research Development Unit (CRDU), Sayad Shirazi Hospital, Golestan University of Medical Sciences, Gorgan, Iran
  9. 9. Stem Cell Research Center, Golestan University of Medical Sciences, Gorgan, Iran
  10. 10. Division of Gastroenterology, Department of Medicine, Duke University, Durham, NC, United States
  11. 11. Department of Immunology, School of Medicine, Tehran University of Medical Science, Tehran, Iran
  12. 12. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  13. 13. Division of Clinical Immunology, Department of Biosciences and Nutrition, Karolinska Institute, Stockholm, Sweden
  14. 14. Department of Neurology, Thomas Jefferson University, Philadelphia, PA, United States

Source: Allergy# Asthma and Clinical Immunology Published:2023


Abstract

Background: Selective IgA deficiency (SIgAD) is the most prevalent inborn errors of immunity with almost unknown etiology. This study aimed to investigate the clinical diagnostic and prognostic values of lymphocyte subsets and function in symptomatic SIgAD patients. Methods: A total of 30 available SIgAD patients from the Iranian registry and 30 age-sex-matched healthy controls were included in the present study. We analyzed B and T cell peripheral subsets and T cell proliferation assay by flow cytometry in SIgAD patients with mild and severe clinical phenotypes. Results: Our results indicated a significant increase in naive and transitional B cells and a strong decrease in marginal zone-like and switched memory B-cells in SIgAD patients. We found that naive and central memory CD4+ T cell subsets, as well as Th1, Th2 and regulatory T cells, have significantly decreased. On the other hand, there was a significant reduction in central and effector memory CD8+ T cell subsets, whereas proportions of both (CD4+ and CD8+) terminally differentiated effector memory T cells (TEMRA) were significantly elevated in our patients. Although some T cell subsets in severe SIgAD were similar, a decrease in marginal-zone and switched memory B cells and an increase in CD21low B cell of severe SIgAD patients were slightly prominent. Moreover, the proliferation activity of CD4+ T cells was strongly impaired in SIgAD patients with a severe phenotype. Conclusion: SIgAD patients have varied cellular and humoral deficiencies. Therefore, T cell and B cell assessment might help in better understanding the heterogeneous pathogenesis and prognosis estimation of the disease. © 2023, The Author(s).
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