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B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency
Common variable immunodeficiency (CVID) is a heterogeneous group of inborn errors of immunity characterized by reduced serum concentrations of different immunoglobulin isotypes. CVID is the most prevalent symptomatic antibody deficiency with a broad range of infectious and non-infectious clinical ma...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9241517/ https://www.ncbi.nlm.nih.gov/pubmed/35784324 http://dx.doi.org/10.3389/fimmu.2022.912826 |
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author | Fekrvand, Saba Khanmohammadi, Shaghayegh Abolhassani, Hassan Yazdani, Reza |
author_facet | Fekrvand, Saba Khanmohammadi, Shaghayegh Abolhassani, Hassan Yazdani, Reza |
author_sort | Fekrvand, Saba |
collection | PubMed |
description | Common variable immunodeficiency (CVID) is a heterogeneous group of inborn errors of immunity characterized by reduced serum concentrations of different immunoglobulin isotypes. CVID is the most prevalent symptomatic antibody deficiency with a broad range of infectious and non-infectious clinical manifestations. Various genetic and immunological defects are known to be involved in the pathogenesis of CVID. Monogenic defects account for the pathogenesis of about 20-50% of CVID patients, while a variety of cases do not have a defined genetic background. Deficiencies in molecules of B cell receptor signaling or other pathways involving B-cell development, activation, and proliferation could be associated with monogenetic defects of CVID. Genetic defects damping different B cell developmental stages can alter B- and even other lymphocytes’ differentiation and might be involved in the clinical and immunologic presentations of the disorder. Reports concerning T and B cell abnormalities have been published in CVID patients, but such comprehensive data on monogenic CVID patients is few and no review article exists to describe the abrogation of lymphocyte subsets in these disorders. Hence, we aimed to review the role of altered B- and T-cell differentiation in the pathogenesis of CVID patients with monogenic defects. |
format | Online Article Text |
id | pubmed-9241517 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-92415172022-06-30 B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency Fekrvand, Saba Khanmohammadi, Shaghayegh Abolhassani, Hassan Yazdani, Reza Front Immunol Immunology Common variable immunodeficiency (CVID) is a heterogeneous group of inborn errors of immunity characterized by reduced serum concentrations of different immunoglobulin isotypes. CVID is the most prevalent symptomatic antibody deficiency with a broad range of infectious and non-infectious clinical manifestations. Various genetic and immunological defects are known to be involved in the pathogenesis of CVID. Monogenic defects account for the pathogenesis of about 20-50% of CVID patients, while a variety of cases do not have a defined genetic background. Deficiencies in molecules of B cell receptor signaling or other pathways involving B-cell development, activation, and proliferation could be associated with monogenetic defects of CVID. Genetic defects damping different B cell developmental stages can alter B- and even other lymphocytes’ differentiation and might be involved in the clinical and immunologic presentations of the disorder. Reports concerning T and B cell abnormalities have been published in CVID patients, but such comprehensive data on monogenic CVID patients is few and no review article exists to describe the abrogation of lymphocyte subsets in these disorders. Hence, we aimed to review the role of altered B- and T-cell differentiation in the pathogenesis of CVID patients with monogenic defects. Frontiers Media S.A. 2022-06-15 /pmc/articles/PMC9241517/ /pubmed/35784324 http://dx.doi.org/10.3389/fimmu.2022.912826 Text en Copyright © 2022 Fekrvand, Khanmohammadi, Abolhassani and Yazdani https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Fekrvand, Saba Khanmohammadi, Shaghayegh Abolhassani, Hassan Yazdani, Reza B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency |
title | B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency |
title_full | B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency |
title_fullStr | B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency |
title_full_unstemmed | B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency |
title_short | B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency |
title_sort | b- and t-cell subset abnormalities in monogenic common variable immunodeficiency |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9241517/ https://www.ncbi.nlm.nih.gov/pubmed/35784324 http://dx.doi.org/10.3389/fimmu.2022.912826 |
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