{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T04:45:50Z","timestamp":1788410750936,"version":"build-2803163510"},"reference-count":51,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,2,18]],"date-time":"2025-02-18T00:00:00Z","timestamp":1739836800000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100016003","name":"Istanbul \u00dcniversitesi-Cerrahpasa","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100016003","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Scand J Immunol"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The group of patients with DNA\u2010repair\u2010defects increases susceptibility to infections due to impaired repertoire diversity. In this context, we aimed to investigate the TCRv\u03b2\u2010repertoire by flow cytometric analysis and its correlation with clinical entities in a group of IEI patients with DNA repair defects. Peripheral lymphocyte subset and TCRv\u03b2\u2010repertoire analyses were performed by flow cytometric analysis. The aim was to explore the changing TCR\u2010V\u03b2\u2010repertoire that can predict some clinical entities by investigating the repertoire using flow\u2010cytometric\u2010analysis\u2010based TCR\u2010V\u03b2 and its interaction with clinical entities in a group of IEI patients with DNA repair defects. TCR\u2010repertoire of the patients with DNA\u2010repair\u2010defects and healthy controls was analysed with flow\u2010cytometer. The potential of flow\u2010cytometric analysis of the TCR repertoire as a practical and easily accessible clinical prediction method was investigated. Thirty\u2010nine\u2010IEI patients with DNA\u2010repair\u2010defects and 15 age\u2010matched healthy\u2010controls were included in this study. Peripheral lymphocyte subset and TCR\u2010V\u03b2 repertoire analyses were performed by flow cytometry. Compared to the control group, 9 out of 24 clones (37.5%) exhibited a statistically significant reduction, while only 3 clones showed a statistically significant increase (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). Preferential use of v\u03b2\u2010genes was associated with some clinical entities. Lower TCR\u2010v\u03b2\u20109 and TCR\u2010v\u03b223, higher TCR\u2010v\u03b27.2 were found in the patients with pneumonia (<jats:italic>n<\/jats:italic>\u2009=\u200913) (<jats:italic>p<\/jats:italic>\u2009=\u20090.018, <jats:italic>p<\/jats:italic>\u2009=\u20090.044 <jats:italic>p<\/jats:italic>\u2009=\u20090.032). AT patients with pneumonia had lower TCR\u2010v\u03b2\u20109 clone than patients without pneumonia (<jats:italic>p<\/jats:italic>\u2009=\u20090.008). Skewed proliferation of most TCR\u2010v\u03b2 clones was seen DNA\u2010repair\u2010defects, especially AT. In addition, this study showed that preferential use of TCR\u2010v\u03b2 genes could be predictive for some clinical entities.<\/jats:p>","DOI":"10.1111\/sji.70003","type":"journal-article","created":{"date-parts":[[2025,2,19]],"date-time":"2025-02-19T06:45:32Z","timestamp":1739947532000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Evaluation of T\u2010cell repertoire by flow cytometric analysis in primary immunodeficiencies with DNA repair defects"],"prefix":"10.1111","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0303-7146","authenticated-orcid":false,"given":"Betul Gemici","family":"Karaaslan","sequence":"first","affiliation":[{"name":"Cerrahpasa School of Medicine, Department of Pediatric Immunology and Allergy Istanbul University\u2010Cerrahpasa  Istanbul 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