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Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report

Pure red-cell aplasia (PRCA) is a syndrome associated with reduced erythroid precursors. This report presents the case of an elderly PRCA patient with significantly proliferated γδT cells and clonal T-cell receptor (TCR) gene rearrangement. The cause of this patient’s PRCA was confirmed to be an aut...

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Autores principales: Li, Xian, Zhu, Xinyi, Zhang, Xiaohong, Wang, Weiqin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9885080/
https://www.ncbi.nlm.nih.gov/pubmed/36726982
http://dx.doi.org/10.3389/fimmu.2022.1103448
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author Li, Xian
Zhu, Xinyi
Zhang, Xiaohong
Wang, Weiqin
author_facet Li, Xian
Zhu, Xinyi
Zhang, Xiaohong
Wang, Weiqin
author_sort Li, Xian
collection PubMed
description Pure red-cell aplasia (PRCA) is a syndrome associated with reduced erythroid precursors. This report presents the case of an elderly PRCA patient with significantly proliferated γδT cells and clonal T-cell receptor (TCR) gene rearrangement. The cause of this patient’s PRCA was confirmed to be an autoimmune disorder rather than malignancy on the basis of flow cytometry, TCR gene rearrangement, and positron emission tomography/computed tomography (PET/CT) findings. Moreover, the γδT cell group identified in this case was captured for the first time under the microscope; this CD4+/CD8− (extremely high CD4/CD8 ratio) population is rare in PRCA patients. Our patient with a monoclonal and polyclonal hybrid of TCR gene rearrangement was sensitive to cyclosporin A (CsA), despite previous reports suggesting that patients with TCR clonal rearrangement may respond poorly to this drug. Overall, this case presents valuable clinical findings for the future diagnosis and management of PRCA caused by autoimmune conditions and further research on γδT cells’ autoimmune pathophysiology and gene rearrangement.
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spelling pubmed-98850802023-01-31 Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report Li, Xian Zhu, Xinyi Zhang, Xiaohong Wang, Weiqin Front Immunol Immunology Pure red-cell aplasia (PRCA) is a syndrome associated with reduced erythroid precursors. This report presents the case of an elderly PRCA patient with significantly proliferated γδT cells and clonal T-cell receptor (TCR) gene rearrangement. The cause of this patient’s PRCA was confirmed to be an autoimmune disorder rather than malignancy on the basis of flow cytometry, TCR gene rearrangement, and positron emission tomography/computed tomography (PET/CT) findings. Moreover, the γδT cell group identified in this case was captured for the first time under the microscope; this CD4+/CD8− (extremely high CD4/CD8 ratio) population is rare in PRCA patients. Our patient with a monoclonal and polyclonal hybrid of TCR gene rearrangement was sensitive to cyclosporin A (CsA), despite previous reports suggesting that patients with TCR clonal rearrangement may respond poorly to this drug. Overall, this case presents valuable clinical findings for the future diagnosis and management of PRCA caused by autoimmune conditions and further research on γδT cells’ autoimmune pathophysiology and gene rearrangement. Frontiers Media S.A. 2023-01-16 /pmc/articles/PMC9885080/ /pubmed/36726982 http://dx.doi.org/10.3389/fimmu.2022.1103448 Text en Copyright © 2023 Li, Zhu, Zhang and Wang 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
Li, Xian
Zhu, Xinyi
Zhang, Xiaohong
Wang, Weiqin
Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report
title Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report
title_full Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report
title_fullStr Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report
title_full_unstemmed Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report
title_short Successful treatment of a pure red-cell aplasia patient with γδT cells and clonal TCR gene rearrangement: A case report
title_sort successful treatment of a pure red-cell aplasia patient with γδt cells and clonal tcr gene rearrangement: a case report
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9885080/
https://www.ncbi.nlm.nih.gov/pubmed/36726982
http://dx.doi.org/10.3389/fimmu.2022.1103448
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