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Lymphocytes Subsets in Correlation With Clinical Profile in Cvid Patients Without Monogenic Defects Publisher Pubmed



Tofighi Zavareh F1, 2, 3 ; Mirshafiey A1, 2 ; Yazdani R1 ; Keshtkar AA4 ; Abolhassani H1, 5 ; Bagheri Y6 ; Rezaei A1 ; Delavari S1 ; Rezaei N1, 3, 7 ; 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 Health Sciences Education Development, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
  6. 6. Clinical Research Development Unit (CRDU), 5 Azar Hospital, Golestan University of Medical Sciences, Gorgan, Iran
  7. 7. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Expert Review of Clinical Immunology Published:2021


Abstract

Objectives: Common variable immunodeficiency (CVID) patients experience clinical manifestations rather than recurrent respiratory infections including autoimmunity, enteropathy, and lymphoproliferation. We evaluated the correlation of lymphocyte subpopulations with such manifestations. Methods: Twenty-six genetically unsolved CVID patients were subdivided into four phenotypes: infection only (IO), autoimmunity (AI), chronic enteropathy (CE), and lymphoproliferative disorders (LP) and examined for lymphocyte subsets by flow cytometry and TCD4+ proliferation by Carboxyfluorescein succinimidyl ester (CFSE) test. Results: We detected reduced memory B and increased total, effector memory (EM), cytotoxic, and activated TCD8+ in IO, AI and CE, decreased plasmablasts, total and naive TCD4+, Regulatory TCD4+ (Treg) and naive TCD8+ in IO and CE, elevated CD21low B and terminally differentiated effector memory (TEMRA) TCD8+ in IO and AI, increased helper T (Th2) and Th17 in IO, decreased Th1 in AI and defective total and naive B and central memory (CM) TCD4+ in CE. IO showed reduced TCD4+ proliferation response. Conclusions: In genetically unsolved CVID patients, increased Th2 and Th17 and reduced Treg is associated with IO, increased CD21low B and TEMRA TCD8+ and reduced Th1 is contributed to AI and reduced total and naive B, CM TCD4+ and naive TCD8+ and expanded total TCD8+ is correlated with CE. © 2021 Informa UK Limited, trading as Taylor & Francis Group.
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