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Role of Rare Immune Cells in Common Variable Immunodeficiency Publisher Pubmed



Soltani M1 ; Rezaei M1 ; Fekrvand S2 ; Ganjalikhanihakemi M1, 3 ; Abolhassani H2, 4, 5 ; Yazdani R2, 6, 7
Authors
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Authors Affiliations
  1. 1. Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
  2. 2. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
  4. 4. Division of Clinical Immunology, Department of Biosciences and Nutrition, Karolinska Institute, Stockholm, Sweden
  5. 5. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
  6. 6. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  7. 7. Department of Neurology, Thomas Jefferson University, Philadelphia, PA, United States

Source: Pediatric Allergy and Immunology Published:2022


Abstract

Common variable immunodeficiency disorder (CVID) is a heterogeneous disorder and the most common symptomatic antibody deficiency disease characterized with hypogammaglobulinemia and a broad range of clinical manifestations. Multiple genetic, epigenetic, and immunological defects are involved in the pathogenesis of CVID. These immunological defects include abnormalities in the number and/or function of B lymphocytes, T lymphocytes, and other rare immune cells. Although some immune cells have a relatively lower proportion among total immune subsets in the human body, they could have important roles in the pathogenesis of immunological disorders like CVID. To the best of our knowledge, this is the first review that described the role of rare immune cells in the pathogenesis and clinical presentations of CVID. © 2021 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.
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