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Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia

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Titel: Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
Autoren: Hermann Kreyenberg, Necil Kutukculer, Barbara Pietrucha, Nermeen Galal, Koen J. van Aerde, Claudio Pignata, Renate Krueger, Gerhard Kindle, Aileen Buecker, Alessandro Plebani, Peter D. Arkwright, Luis Ignacio Gonzalez Granado, E. Graham Davies, Markus G. Seidel, Ruth Pia Duecker, Ulrich Baumann, Nizar Mahlaoui, Peter Bader, Stephan Ehl, Sandra Woelke, Peter Ciznar, Tim Niehues, Hans-Juergen Laws, Sabine Huenecke, Shereen M. Reda, Ismail Reisli, Larysa Kostyuchenko, Stefan Zielen, Michiel van der Flier, Juan Luis Santos Pérez, Sara Sebnem Kilic, Laura Hora Marques, Maria Kanariou, Carlos Rodríguez-Gallego, Pere Soler-Palacín, Isabelle Meyts, Bodo Grimbacher, Catharina Schuetz, Dagmar Graf, E.V. Deripapa, Claire Bethune, Seraina Prader, Kai Lehmberg, Elizabeth M. McDermott, Fabian Hauck, John David M Edgar, Sharhzad Bakhtiar, Horst von Bernuth, Sabine M El-Helou, Helena Donath, Svetlana O. Sharapova, Ralf Schubert, Mary Slatter
Weitere Verfasser: Universitat Autònoma de Barcelona, Institut Català de la Salut, [Zielen S, Duecker RP, Woelke S, Donath H, Buecker A] Division of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. [Bakhtiar S] Division for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. [Soler-Palacín P] Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Unitat de Patologia Infecciosa i Immunodeficiències de Pediatria, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain, Vall d'Hebron Barcelona Hospital Campus
Quelle: J Clin Immunol
Dipòsit Digital de Documents de la UAB
Universitat Autònoma de Barcelona
Scientia
Scientia. Dipòsit d'Informació Digital del Departament de Salut
instname
Repositorio Institucional de la Consejería de Sanidad de la Comunidad de Madrid
Consejería de Sanidad de la Comunidad de Madrid
RISalud-ANDALUCIA. Repositorio Institucional de Salud de Andalucía
Journal of Clinical Immunology, 41, 8, pp. 1878-1892
Verlagsinformationen: Springer Science and Business Media LLC, 2021.
Publikationsjahr: 2021
Schlagwörter: Male, 0301 basic medicine, Epidemiology, Atàxia de Friedreich - Prognosi, PHENOTYPE, Ataxia-telangiectasia, T-Lymphocyte Subsets, T-CELL SUBSETS, Repertoire, Paediatrics - Radboud University Medical Center, OXIDATIVE STRESS, IgG Deficiency, Child, Internal medicine, Radboudumc 4: lnfectious Diseases and Global Health RIMLS: Radboud Institute for Molecular Life Sciences, Immunology and Microbiology, ddc:610, IMMUNODEFICIENCY, B-Lymphocytes, 0303 health sciences, ERN-RITA, Jeffrey Modell foundation, IgA Deficiency, Life Sciences, Middle Aged, Immunoglobulina A, 3. Good health, DEFICIENCY, 3204 Immunology, ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::enfermedades cerebelosas::ataxia cerebelosa::ataxias espinocerebelosas::ataxia telangiectasia, Phenotype, INFECTIONS, 1107 Immunology, T-Cell Subsets, Child, Preschool, Deficiency, Medicine, IgA deficiency, Original Article, Female, Life Sciences & Biomedicine, Receptor, Adult, Adolescent, DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebellar Diseases::Cerebellar Ataxia::Spinocerebellar Ataxias::Ataxia Telangiectasia, Hypogammaglobulinemia, Immunology, Generation, Immunoglobulins, Ataxia Telangiectasia/mortality [MeSH], Immunoglobulin A/blood [MeSH], T-Lymphocyte Subsets/immunology [MeSH], Lymphopenia, Infant [MeSH], Mortality, Male [MeSH], Ataxia Telangiectasia/immunology [MeSH], Child [MeSH], Adolescent [MeSH], Female [MeSH], IgG Deficiency/immunology [MeSH], Immunodeficiency, Immunoglobulin M/blood [MeSH], Adult [MeSH], B-Lymphocytes/immunology [MeSH], Lymphocyte Count [MeSH], Humans [MeSH], Middle Aged [MeSH], IgA Deficiency/immunology [MeSH], Young Adult [MeSH], IgA Deficiency/mortality [MeSH], Immunoglobulin G/blood [MeSH], IgG Deficiency/mortality [MeSH], Child, Preschool [MeSH], Infections, REPERTOIRE, Ataxia Telangiectasia, Young Adult, 03 medical and health sciences, Epidemiology and Management of Cytomegalovirus Infection, Biochemistry, Genetics and Molecular Biology, Health Sciences, Genetics, Humans, Lymphocyte Count, Other subheadings::Other subheadings::Other subheadings::/immunology, Molecular Biology, Biology, Antibody, Immune Evasion, Science & Technology, RECEPTOR, Genetic Basis of Primary Immunodeficiency Disorders, FOS: Clinical medicine, Immunity, Infant, CD8, DNA, Immunoglobulin A, Molecular Mechanisms of DNA Damage Response, Oxidative Stress, Otros calificadores::Otros calificadores::Otros calificadores::/inmunología, Immune system, Immunoglobulin M, Atm, ATM, Immunoglobulin G, FOS: Biological sciences, DNA damage, GENERATION
Beschreibung: Patients with ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. From a clinical point of view, A-T patients with IgA deficiency show more symptoms and may have a poorer prognosis. In this study, we analyzed mortality and immunity data of 659 A-T patients with regard to IgA deficiency collected from the European Society for Immunodeficiencies (ESID) registry and from 66 patients with classical A-T who attended at the Frankfurt Goethe-University between 2012 and 2018. We studied peripheral B- and T-cell subsets and T-cell repertoire of the Frankfurt cohort and survival rates of all A-T patients in the ESID registry. Patients with A-T have significant alterations in their lymphocyte phenotypes. All subsets (CD3, CD4, CD8, CD19, CD4/CD45RA, and CD8/CD45RA) were significantly diminished compared to standard values. Patients with IgA deficiency (n = 35) had significantly lower lymphocyte counts compared to A-T patients without IgA deficiency (n = 31) due to a further decrease of naïve CD4 T-cells, central memory CD4 cells, and regulatory T-cells. Although both patient groups showed affected TCR-ß repertoires compared to controls, no differences could be detected between patients with and without IgA deficiency. Overall survival of patients with IgA deficiency was significantly diminished. For the first time, our data show that patients with IgA deficiency have significantly lower lymphocyte counts and subsets, which are accompanied with reduced survival, compared to A-T patients without IgA deficiency. IgA, a simple surrogate marker, is indicating the poorest prognosis for classical A-T patients. Both non-interventional clinical trials were registered at clinicaltrials.gov 2012 (Susceptibility to infections in ataxia-telangiectasia; NCT02345135) and 2017 (Susceptibility to Infections, tumor risk and liver disease in patients with ataxia-telangiectasia; NCT03357978)
Publikationsart: Article
Other literature type
Dateibeschreibung: application/pdf; application/octet-stream; pdf
Sprache: English
ISSN: 1573-2592
0271-9142
DOI: 10.1007/s10875-021-01090-8
DOI: 10.60692/f9tmv-1k425
DOI: 10.60692/k31ge-sre35
Zugangs-URL: https://link.springer.com/content/pdf/10.1007/s10875-021-01090-8.pdf
https://pubmed.ncbi.nlm.nih.gov/34477998
https://ddd.uab.cat/record/250449
https://hdl.handle.net/11351/7788
https://hdl.handle.net/20.500.12530/63950
http://hdl.handle.net/10668/18493
https://hdl.handle.net/https://repository.ubn.ru.nl/handle/2066/241476
https://link.springer.com/content/pdf/10.1007/s10875-021-01090-8.pdf
https://eprints.ncl.ac.uk/277024
https://www.ncbi.nlm.nih.gov/pubmed/34477998
https://pubmed.ncbi.nlm.nih.gov/34477998/
https://avesis.ege.edu.tr/yayin/030415c1-efab-40d9-93cb-54c478ac71d8/simple-measurement-of-iga-predicts-immunity-and-mortality-in-ataxia-telangiectasia
https://link.springer.com/article/10.1007/s10875-021-01090-8
https://lirias.kuleuven.be/handle/123456789/681542
https://doi.org/10.1007/s10875-021-01090-8
https://hdl.handle.net/2066/241476
https://repository.ubn.ru.nl//bitstream/handle/2066/241476/241476.pdf
https://hdl.handle.net/10668/18493
https://repository.publisso.de/resource/frl:6446037
https://avesis.uludag.edu.tr/publication/details/030415c1-efab-40d9-93cb-54c478ac71d8/oai
http://publikationen.ub.uni-frankfurt.de/files/63573/container.zip
Rights: CC BY
URL: http://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (http://creativecommons.org/licenses/by/4.0/) .
Dokumentencode: edsair.doi.dedup.....70c067cc4e015b3d0afd59278267b96f
Datenbank: OpenAIRE
Beschreibung
Abstract:Patients with ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. From a clinical point of view, A-T patients with IgA deficiency show more symptoms and may have a poorer prognosis. In this study, we analyzed mortality and immunity data of 659 A-T patients with regard to IgA deficiency collected from the European Society for Immunodeficiencies (ESID) registry and from 66 patients with classical A-T who attended at the Frankfurt Goethe-University between 2012 and 2018. We studied peripheral B- and T-cell subsets and T-cell repertoire of the Frankfurt cohort and survival rates of all A-T patients in the ESID registry. Patients with A-T have significant alterations in their lymphocyte phenotypes. All subsets (CD3, CD4, CD8, CD19, CD4/CD45RA, and CD8/CD45RA) were significantly diminished compared to standard values. Patients with IgA deficiency (n = 35) had significantly lower lymphocyte counts compared to A-T patients without IgA deficiency (n = 31) due to a further decrease of naïve CD4 T-cells, central memory CD4 cells, and regulatory T-cells. Although both patient groups showed affected TCR-ß repertoires compared to controls, no differences could be detected between patients with and without IgA deficiency. Overall survival of patients with IgA deficiency was significantly diminished. For the first time, our data show that patients with IgA deficiency have significantly lower lymphocyte counts and subsets, which are accompanied with reduced survival, compared to A-T patients without IgA deficiency. IgA, a simple surrogate marker, is indicating the poorest prognosis for classical A-T patients. Both non-interventional clinical trials were registered at clinicaltrials.gov 2012 (Susceptibility to infections in ataxia-telangiectasia; NCT02345135) and 2017 (Susceptibility to Infections, tumor risk and liver disease in patients with ataxia-telangiectasia; NCT03357978)
ISSN:15732592
02719142
DOI:10.1007/s10875-021-01090-8