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Journal of Clinical Immunology
Published in: Journal of Clinical Immunology 3/2020

01-04-2020 | Trisomy 21 | Original Article

A Prevalent CXCR3+ Phenotype of Circulating Follicular Helper T Cells Indicates Humoral Dysregulation in Children with Down Syndrome

Authors: Giorgio Ottaviano, Jolanda Gerosa, Micaela Santini, Pasqualina De Leo, Andrea Vecchione, Tatiana Jofra, Cristiana Trimarchi, Maurizio De Pellegrin, Massimo Agosti, Alessandro Aiuti, Maddalena Marinoni, Maria Pia Cicalese, Georgia Fousteri

Published in: Journal of Clinical Immunology | Issue 3/2020

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Abstract

Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of children with DS. Blood was collected from 24 children with DS, nine of which had autoimmune diseases. Children with DS showed skewed Tfh differentiation towards the CXCR3+ phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3+PD-1+ subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3+ phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients.
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Metadata
Title
A Prevalent CXCR3+ Phenotype of Circulating Follicular Helper T Cells Indicates Humoral Dysregulation in Children with Down Syndrome
Authors
Giorgio Ottaviano
Jolanda Gerosa
Micaela Santini
Pasqualina De Leo
Andrea Vecchione
Tatiana Jofra
Cristiana Trimarchi
Maurizio De Pellegrin
Massimo Agosti
Alessandro Aiuti
Maddalena Marinoni
Maria Pia Cicalese
Georgia Fousteri
Publication date
01-04-2020
Publisher
Springer US
Keyword
Trisomy 21
Published in
Journal of Clinical Immunology / Issue 3/2020
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-020-00755-0

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