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The Heterogeneous Pathogenesis of Selective Immunoglobulin a Deficiency Publisher Pubmed



Bagheri Y1, 2, 3 ; Sanaei R4, 5 ; Yazdani R2 ; Shekarabi M3, 4 ; Falak R3, 4 ; Mohammadi J6 ; Abolhassani H2, 7 ; Aghamohammadi A2
Authors
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Authors Affiliations
  1. 1. Clinical Research Development Unit (CRDU), 5 Azar Hospital, Golestan University of Medical Sciences, Gorgan, Iran
  2. 2. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, 62 Qarib Street, Keshavarz Blvd., Tehran, 14194, Iran
  3. 3. Department of Immunology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
  4. 4. Immunology Research Center (IRC), Institute of Immunology and Infectious Diseases, Iran University of Medical Sciences, Tehran, Iran
  5. 5. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  6. 6. Department of Biomedical Engineering, Faculty of New Sciences and Technologies, University of Tehran, Tehran, Iran
  7. 7. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden

Source: International Archives of Allergy and Immunology Published:2019


Abstract

Selective immunoglobulin A deficiency (SIgAD) is the most prevalent type of primary immunodeficiency disorder. The phenotypic feature of SIgAD is related to a defect in B lymphocyte differentiation into plasma cell-producing immunoglobulin A (IgA). In this review, we summarize the recent advances in this regard. Genetic (including major histocompatibility complex [MHC] and non-MHC genes), immunologic (including B and T lymphocyte subsets abnormality), cytokines/chemokines and their related receptors, apoptosis and microbiota defects are reviewed. The mechanisms leading to SIgAD are most likely multifactorial and it can be speculated that several pathways controlling B cells functions or regulating epigenetic of the IGHA gene encoding constant region of IgA heavy chain and long-term survival of IgA switched memory B cells and plasma cells may be defective in different SIgAD patients. © 2019 S. Karger AG, Basel.
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