ArticleEuropean journal of immunology2022
Follicular helper T cell signature of replicative exhaustion, apoptosis, and senescence in common variable immunodeficiency.
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Article in European journal of immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 10 papers.
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10citing papers in PubMed
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Who cites it
10 citing papers in PubMed, 17 citations in OpenAlex.
- Beyond B cells: T and Innate Immune Mechanisms of Autoimmunity in Common Variable Immunodeficiency.Clinical reviews in allergy & immunology · 2026Review
- Clinical and immunological differentiation of isolated IgG and combined IgG & IgM deficiencies from common variable immunodeficiency.Frontiers in immunology · 2026Article
- T follicular helper cells in primary immune regulatory disorders.The Journal of allergy and clinical immunology · 2025Review
- CXCL13 as a Biomarker of Complex Common Variable Immunodeficiency.Journal of clinical immunology · 2025Article
- Emergence of a senescent and inflammatory pulmonary CD4Science advances · 2025Article
- STAT expression and TFH1 cells in CVID granulomatosis and sarcoidosis: immunological and histopathological comparisons.Virchows Archiv : an international journal of pathology · 2024Article
- Article
- Review
- Impaired B Cell Recall Memory and Reduced Antibody Avidity but Robust T Cell Response in CVID Patients After COVID-19 Vaccination.Journal of clinical immunology · 2023Article
- Natural history of type 1 diabetes on an immunodysregulatory background with genetic alteration in B-cell activating factor receptor: A case report.Frontiers in immunology · 2022Article
Corrections and comments
- Erratum issuedCorrection.2023
Authors and funding
54 authors at 15 institutions in 2 countries.
Giulia MilardiDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-5401-5377
Biagio Di LorenzoDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-6030-2583
Jolanda GerosaDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0001-6469-7378
Federica BarzaghiPediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-4498-1712
Gigliola Di MatteoDepartment of Systems Medicine, University of Rome Tor Vergata, Via Cracovia 50, Rome, 00133, Italy.ORCID 0000-0001-8344-5635
Maryam OmraniPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-0056-109X
Tatiana JofraDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-2825-155X
Ivan MerelliPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-3587-3680
Matteo BarcellaPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-2353-3999
Matteo FilippiniDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-0730-4088
Anastasia ContiPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-8670-2649
Francesca FerruaPediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-5695-4490
Francesco Pozzo GiuffridaPediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-2309-8157
Francesca DionisioPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-0999-2627
Patrizia Rovere-QueriniDepartment of Immunology, Transplantation and Infectious Diseases, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-2615-3649
Sarah MarktelHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-3768-4288
Andrea AssanelliHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.
Simona PiemonteseHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-5908-0792
Immacolata BrigidaPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-0338-2230
Matteo ZoccolilloPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0001-6994-8455
Emilia CirilloDepartment of Translational Medical Sciences, Section of Pediatrics, Federico II University of Naples, Corso Umberto I, 40, 80138, Italy.ORCID 0000-0002-0605-649X
Giuliana GiardinoDepartment of Translational Medical Sciences, Section of Pediatrics, Federico II University of Naples, Corso Umberto I, 40, 80138, Italy.ORCID 0000-0002-0621-9527
Maria Giovanna DanieliDepartment of Clinical and Molecular Sciences, Marche Polytechnic University of Ancona, Clinica Medica, Via Tronto 10/a, Ancona, 60126, Italy.ORCID 0000-0002-9608-2252
Fernando SpecchiaDepartment of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Via Giuseppe Massarenti 9, Bologna, 40138, Italy.ORCID 0000-0002-6411-0888
Lucia PacilloDepartment of Systems Medicine, University of Rome Tor Vergata, Via Cracovia 50, Rome, 00133, Italy.ORCID 0000-0002-7950-8848
Silvia Di CesareDepartment of Systems Medicine, University of Rome Tor Vergata, Via Cracovia 50, Rome, 00133, Italy.ORCID 0000-0002-9912-7997
Carmela GiancottaDepartment of Systems Medicine, University of Rome Tor Vergata, Via Cracovia 50, Rome, 00133, Italy.ORCID 0000-0003-0387-5593
Francesca RomanoPediatric Immunology Division, Department of Pediatrics, Anna Meyer Children's University Hospital, Viale Gaetano Pieraccini 24, Florence, 50139, Italy.ORCID 0000-0001-5651-157X
Alessandro MatareseDepartment of Respiratory Medicine, Santi Antonio, Biagio and Cesare Arrigo Hospital, Via Venezia 16, Alessandria, 15121, Italy.
Alfredo Antonio ChettaDepartment of Medicine and Surgery, Respiratory Disease and Lung Function Unit, University of Parma, Str. dell'Università 12, Parma, 43121, Italy.ORCID 0000-0001-8449-368X
Matteo TrimarchiOtorhinolaryngology Unit, Head and Neck Department, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0001-6472-3484
Andrea LaurenziDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-6073-5098
Maurizio De PellegrinUnit of Orthopaedics, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-8376-7420
Silvia DarinPediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.
Davide MontinDepartment of Pediatrics and Public Health, Regina Margherita Hospital, Piazza Polonia 94, Turin, 10126, Italy.ORCID 0000-0001-8953-2182
Maddalena MarinoniPediatric Unit, Ospedale "F. Del Ponte", Via Filippo del Ponte 19, Varese, 21100, Italy.ORCID 0000-0003-2520-9137
Rosa Maria DellepianeDepartment of Pediatrics, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, University of Milan, Via Francesco Sforza 35, Milan, 20122, Italy.ORCID 0000-0002-9921-3414
Valeria SordiDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-0179-1679
Vassilios LougarisDepartment of Clinical and Experimental Sciences, Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, University of Brescia, Piazza del Mercato 15, Brescia, 25121, Italy.ORCID 0000-0003-2303-9533
Angelo VaccaDepartment of Biomedical Sciences and Human Oncology, University of Bari Medical School, Piazza Umberto I, 1, Bari, 70121, Italy.ORCID 0000-0002-4567-8216
Raffaella MelziDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-1511-8266
Rita NanoDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-4520-8481
Chiara AzzariPediatric Immunology Division, Department of Pediatrics, Anna Meyer Children's University Hospital, Viale Gaetano Pieraccini 24, Florence, 50139, Italy.ORCID 0000-0001-7236-1655
Lucia BongiovanniPathology Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-3558-2771
Claudio PignataDepartment of Translational Medical Sciences, Section of Pediatrics, Federico II University of Naples, Corso Umberto I, 40, 80138, Italy.ORCID 0000-0003-1568-9843
Caterina CancriniDepartment of Systems Medicine, University of Rome Tor Vergata, Via Cracovia 50, Rome, 00133, Italy.ORCID 0000-0001-8410-9617
Alessandro PlebaniDepartment of Clinical and Experimental Sciences, Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, University of Brescia, Piazza del Mercato 15, Brescia, 25121, Italy.ORCID 0000-0002-2003-3307
Lorenzo PiemontiDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-2172-2198
Constantinos PetrovasTissue Analysis Core, Immunology Laboratory, Vaccine Research Center, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD, 20892, USA.ORCID 0000-0002-5067-5810
Raffaella Di MiccoPathogenesis and therapy of primary immunodeficiencies Unit, San Raffaele Telethon Institute for Gene Therapy, Sr-TIGET, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-0639-8238
Maurilio PonzoniPathology Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-0055-325X
Alessandro AiutiPediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0002-5398-1717
Maria Pia CicalesePediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0003-0371-3279
Georgia FousteriDivision of Immunology, Transplantation, and Infectious Diseases, Diabetes Research Institute, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy.ORCID 0000-0001-7745-7517
Vita-Salute San Raffaele University · ITThe San Raffaele Telethon Institute for Gene Therapy · ITUniversity of Rome Tor Vergata · ITFederico II University Hospital · ITMeyer Children's Hospital · ITUniversity of Brescia · ITAzienda Ospedaliera Nazionale SS. Antonio e Biagio e Cesare Arrigo · ITIRCCS Azienda Ospedliero-Universitaria di Bologna Policlinico di Sant'Orsola · ITMarche Polytechnic University · ITNational Institutes of Health · USOspedale Filippo Del Ponte Varese · ITOspedale Regina Margherita · ITUniversity of Bari Aldo Moro · ITUniversity of Milan · ITUniversity of Parma · IT
Funding
No grant is acknowledged in the PubMed record.
Abstract
Common variable immunodeficiency (CVID) is the most frequent primary antibody deficiency whereby follicular helper T (Tfh) cells fail to establish productive responses with B cells in germinal centers. Here, we analyzed the frequency, phenotype, transcriptome, and function of circulating Tfh (cTfh) cells in CVID patients displaying autoimmunity as an additional phenotype. A group of patients showed a high frequency of cTfh1 cells and a prominent expression of PD-1 and ICOS as well as a cTfh mRNA signature consistent with highly activated, but exhausted, senescent, and apoptotic cells. Plasmatic CXCL13 levels were elevated in this group and positively correlated with cTfh1 cell frequency and PD-1 levels. Monoallelic variants in RTEL1, a telomere length- and DNA repair-related gene, were identified in four patients belonging to this group. Their blood lymphocytes showed shortened telomeres, while their cTfh were more prone to apoptosis. These data point toward a novel pathogenetic mechanism in CVID, whereby alterations in DNA repair and telomere elongation might predispose to antibody deficiency. A Th1, highly activated but exhausted and apoptotic cTfh phenotype was associated with this form of CVID.
Indexed as
Common Variable ImmunodeficiencyApoptosisHumansProgrammed Cell Death 1 ReceptorT Follicular Helper CellsT-Lymphocytes, Helper-InducerProgrammed Cell Death 1 ReceptorB cellsCommon variable immunodeficiencyImmune agingT-cell exhaustionT follicular helper cells
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