Lymphocyte Subgroups and KREC Numbers in Common Variable Immunodeficiency: A Single Center Study

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dc.authoridNG, YUK YIN/0000-0001-9755-6045|Ozbek Cicek, Begum/0000-0002-9053-2785|Yaz, Ismail/0000-0001-5278-7456|Tan, Cagman/0000-0001-6972-1349|Cagdas Ayvaz, Deniz Nazire/0000-0003-2213-4627|Cagdas Ayvaz, Deniz Nazire/0000-0003-2213-4627|Kosukcu, Can/0000-0002-6129-0510
dc.authorwosidNG, YUK YIN/AAG-7977-2020
dc.authorwosidOzbek Cicek, Begum/AAW-3575-2020
dc.authorwosidYaz, Ismail/AAW-3570-2020
dc.authorwosidGür Çetinkaya, Pınar/JAE-9737-2023
dc.authorwosidTan, Cagman/ABA-2566-2021
dc.authorwosidCagdas Ayvaz, Deniz Nazire/I-9104-2013
dc.authorwosidÇetinkaya, Pınar Gür/AAY-9310-2020
dc.contributor.authorYaz, Ismail
dc.contributor.authorOzbek, Begum
dc.contributor.authorNg, Yuk Yin
dc.contributor.authorCetinkaya, Pinar Gur
dc.contributor.authorHalacli, Sevil Oskay
dc.contributor.authorTan, Cagman
dc.contributor.authorKasikci, Merve
dc.date.accessioned2024-07-18T20:42:13Z
dc.date.available2024-07-18T20:42:13Z
dc.date.issued2020
dc.departmentİstanbul Bilgi Üniversitesien_US
dc.description.abstractCommon variable immunodeficiency (CVID) results in defective B cell differentiation and impaired antibody production and is the most common symptomatic primary immunodeficiency. Our aim was to evaluate the correlation among B cell subgroups, kappa-deleting recombination excision circle (KREC) copy numbers, and clinical and immunological data of the patients with CVID, and evaluate the patients according to classifications currently available to define the role of KREC copy numbers in the diagnosis of CVID. KREC analysis was performed using a quantitative real-time polymerase chain reaction assay, and B cell subgroups were measured by flow cytometry. The median age of the patients (n = 30) was 25 (6-69) years. Parental consanguinity ratio was 33%. The median age at diagnosis was 15 (4-59), and follow-up period was 6 (1-37) years. CD19(+) and CD4(+) cell counts at the time of diagnosis were low in 66.7% and 46.7% of the patients, respectively. CD19(+) cell counts were positively correlated with KREC copy numbers in patients and healthy controls. CD19(+) cell counts and KREC copy numbers were significantly reduced in CVID patients compared to healthy controls as expected. KRECs are quantitative markers for B cell defects. We found low CD4(+) cell numbers, recent thymic emigrants, and lymphopenia in some of the patients at diagnosis, which reminds the heterogeneity of CVID's etiology. In this study, a positive correlation was shown between CD19(+) cell counts and KREC copy numbers. Low KREC copy numbers indicated B cell deficiency; however, high KREC copy numbers were not sufficient to rule out CVID.en_US
dc.description.sponsorshipScientific and Technological Research Council of Turkey [315S125]; Hacettepe University Scientific Research Projects Coordination Unit [N17128]en_US
dc.description.sponsorshipWe appreciate Dr. Luisa Imberti and Dr. Alessandra Sottini from Laboratorio CREA, Spedali Civili di Brescia, Italy, for kindly supplying the TREC-KREC-TCRAC plasmid to our laboratory. The authors thank to the patients and their families for their collaboration and participation. This project was supported by Scientific and Technological Research Council of Turkey (Project Number: 315S125) and also by Hacettepe University Scientific Research Projects Coordination Unit (Project number: N17128).en_US
dc.identifier.doi10.1007/s10875-020-00761-2
dc.identifier.endpage502en_US
dc.identifier.issn0271-9142
dc.identifier.issn1573-2592
dc.identifier.issue3en_US
dc.identifier.pmid32056073en_US
dc.identifier.scopus2-s2.0-85079732083en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage494en_US
dc.identifier.urihttps://doi.org/10.1007/s10875-020-00761-2
dc.identifier.urihttps://hdl.handle.net/11411/7183
dc.identifier.volume40en_US
dc.identifier.wosWOS:000516082100001en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringer/Plenum Publishersen_US
dc.relation.ispartofJournal of Clinical Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectCvıden_US
dc.subjectKrecsen_US
dc.subjectB Cellsen_US
dc.subjectQrt-Pcren_US
dc.subjectFlow Cytometryen_US
dc.subjectT-Cell-Receptoren_US
dc.subjectRecombination Excision Circlesen_US
dc.subjectReplication Historyen_US
dc.subjectClassificationen_US
dc.subjectDeficiencyen_US
dc.subjectDeaminaseen_US
dc.titleLymphocyte Subgroups and KREC Numbers in Common Variable Immunodeficiency: A Single Center Study
dc.typeArticle

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