The evaluation of malignancies in Turkish primary immunodeficiency patients; A multicenter study

dc.contributor.authorMetin, Ayşe
dc.contributor.authorAytekin, Caner
dc.contributor.authorKaraca, Neslihan Edeer
dc.contributor.authorBarış, Safa
dc.contributor.authorKıykım, Ayça
dc.contributor.authorAydıner, Elif Karakoç
dc.contributor.authorÖzen, Ahmet
dc.contributor.authorAksu, Güzide
dc.contributor.authorKütükçüler, Necil
dc.contributor.buuauthorÇekiç, Şükrü
dc.contributor.buuauthorKaralı, Yasin
dc.contributor.buuauthorAslan, Törehan
dc.contributor.buuauthorSevinir, Betül
dc.contributor.buuauthorKılıç, Sara Şebnem
dc.contributor.departmentBursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları.tr_TR
dc.contributor.orcid0000-0002-9574-1842tr_TR
dc.contributor.orcid0000-0002-3966-4635tr_TR
dc.contributor.orcid0000-0003-4150-5200tr_TR
dc.contributor.researcheridFFS-1974-2022tr_TR
dc.contributor.researcheridAAH-1570-2021tr_TR
dc.contributor.scopusid56117061000tr_TR
dc.contributor.scopusid57188056500tr_TR
dc.contributor.scopusid49863694000tr_TR
dc.contributor.scopusid6603199915tr_TR
dc.contributor.scopusid34975059200tr_TR
dc.date.accessioned2023-02-06T08:25:24Z
dc.date.available2023-02-06T08:25:24Z
dc.date.issued2020-02-14
dc.description.abstractBackground There are no data regarding the prevalence of malignancies in patients with primary immunodeficiency (PID) in Turkey. Along with the prevalence of malignancy, we aimed to present the types of malignancy and define the underlying immune deficiency of the patients. Method Between the years 1992 and 2018, from five tertiary immunology clinics, fifty-nine patients with PID who developed malignancy were included. All patients were evaluated for demographics, clinical features, and prognosis. Results The prevalence of malignancy in our cohort was detected as 0.9% (59/6392). The male-to-female ratio was 1.8 (38/21), and the median age of patients was 14 years (range: 1.5-51). The median age at diagnosis of malignancy was 10 years (range: 1.5-51). Ataxia-telangiectasia was the most frequent PID in patients with malignancy (n = 19, 32.2%), and non-Hodgkin lymphoma was the most common malignancy (n = 32, 51.6%). The rate of malignancy in DOCK8 deficiency (n = 7/43, 16.3%) was higher than AT (n = 19/193, 9.8%), Wiskott-Aldrich syndrome (n = 2/22, 9.1%), and common variable immunodeficiency (n = 11/205, 5.4%). EBV quantitative PCR was positive in 16 out of 53 patients (30.2%). Three patients had secondary malignancies. Remission was achieved in 26 patients (44.1%). However, 31 patients (52.5%) died. Two patients (3.4%) are still on chemotherapy. Conclusion This study is the largest cohort investigating the association of malignancy in patients with PID in Turkey. While lymphoid malignancies were the most common malignancy and observed more frequently in AT patients, the risk for malignancy was higher in patients with DOCK8 deficiency compared to AT.en_US
dc.identifier.citationÇekiç, Ş. vd. (2020). "The evaluation of malignancies in Turkish primary immunodeficiency patients; A multicenter study". Pediatric Allergy and Immunology, 31(5), 528-536.en_US
dc.identifier.endpage536tr_TR
dc.identifier.issn0905-6157
dc.identifier.issn1399-3038
dc.identifier.issue5tr_TR
dc.identifier.pubmed32060950tr_TR
dc.identifier.scopus2-s2.0-85081679990tr_TR
dc.identifier.startpage528tr_TR
dc.identifier.urihttps://doi.org/10.1111/pai.13231
dc.identifier.urihttps://onlinelibrary.wiley.com/doi/10.1111/pai.13231
dc.identifier.urihttp://hdl.handle.net/11452/30851
dc.identifier.volume31tr_TR
dc.identifier.wos000550050500001tr_TR
dc.indexed.pubmedPubMeden_US
dc.indexed.scopusScopusen_US
dc.indexed.wosSCIEen_US
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.collaborationYurt içitr_TR
dc.relation.collaborationSanayitr_TR
dc.relation.journalPediatric Allergy and Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergitr_TR
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAllergyen_US
dc.subjectImmunologyen_US
dc.subjectPediatricsen_US
dc.subjectAtaxia-telangiectasiaen_US
dc.subjectCanceren_US
dc.subjectDOCK8 deficiencyen_US
dc.subjectLymphomaen_US
dc.subjectMalignancyen_US
dc.subjectNon-Hodgkin lymphomaen_US
dc.subjectPrimary immunodeficiencyen_US
dc.subjectCancer incidenceen_US
dc.subjectAtaxia-telangiectasiaen_US
dc.subjectLymphomaen_US
dc.subjectPathophysiologyen_US
dc.subjectSecondaryen_US
dc.subjectDiseasesen_US
dc.subjectGeneen_US
dc.subject.emtreeAntineoplastic agenten_US
dc.subject.emtreeDOCK8 proteinen_US
dc.subject.emtreeHumanen_US
dc.subject.emtreeGuanine nucleotide exchange factoren_US
dc.subject.emtreeAcute myeloid leukemiaen_US
dc.subject.emtreeAdolescenten_US
dc.subject.emtreeAgeen_US
dc.subject.emtreeArticleen_US
dc.subject.emtreeAtaxia telangiectasiaen_US
dc.subject.emtreeB cell lymphomaen_US
dc.subject.emtreeBloom syndromeen_US
dc.subject.emtreeBurkitt lymphomaen_US
dc.subject.emtreeCancer chemotherapyen_US
dc.subject.emtreeCancer radiotherapyen_US
dc.subject.emtreeClinical featureen_US
dc.subject.emtreeCohort analysisen_US
dc.subject.emtreeColon adenocarcinomaen_US
dc.subject.emtreeCombined immunodeficiencyen_US
dc.subject.emtreeCommon variable immunodeficiencyen_US
dc.subject.emtreeConsanguineous marriageen_US
dc.subject.emtreeFamily historyen_US
dc.subject.emtreeFemaleen_US
dc.subject.emtreeHemangiopericytomaen_US
dc.subject.emtreeHematologic malignancyen_US
dc.subject.emtreeHematopoietic stem cell transplantationen_US
dc.subject.emtreeHepatosplenomegalyen_US
dc.subject.emtreeHodgkin diseaseen_US
dc.subject.emtreeHumanen_US
dc.subject.emtreeHumoral immune deficiencyen_US
dc.subject.emtreeImmune deficiencyen_US
dc.subject.emtreeLymphadenopathyen_US
dc.subject.emtreeMajor clinical studyen_US
dc.subject.emtreeMaleen_US
dc.subject.emtreeMalignant neoplasmen_US
dc.subject.emtreeMortality rateen_US
dc.subject.emtreeMulticenter studyen_US
dc.subject.emtreeNephroblastomaen_US
dc.subject.emtreeNeurilemomaen_US
dc.subject.emtreeNonhodgkin lymphomaen_US
dc.subject.emtreePriority journalen_US
dc.subject.emtreePrognosisen_US
dc.subject.emtreeReal time polymerase chain reactionen_US
dc.subject.emtreeRectum carcinomaen_US
dc.subject.emtreeRemissionen_US
dc.subject.emtreeSex differenceen_US
dc.subject.emtreeSmall cell sarcomaen_US
dc.subject.emtreeSolid malignant neoplasmen_US
dc.subject.emtreeSquamous cell carcinomaen_US
dc.subject.emtreeT cell lymphomaen_US
dc.subject.emtreeThyroid papillary carcinomaen_US
dc.subject.emtreeTurkey (republic)en_US
dc.subject.emtreeWiskott Aldrich syndromeen_US
dc.subject.emtreeAdulten_US
dc.subject.emtreeAtaxia telangiectasiaen_US
dc.subject.emtreeChilden_US
dc.subject.emtreeClinical trialen_US
dc.subject.emtreeInfanten_US
dc.subject.emtreeMiddle ageden_US
dc.subject.emtreeNeoplasmen_US
dc.subject.emtreePreschool childen_US
dc.subject.emtreeTurkey (bird)en_US
dc.subject.emtreeYoung adulten_US
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshAtaxia telangiectasiaen_US
dc.subject.meshChilden_US
dc.subject.meshChild, preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshGuanine nucleotide exchange factorsen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunologic deficiency syndromesen_US
dc.subject.meshInfanten_US
dc.subject.meshMaleen_US
dc.subject.meshMiddle ageden_US
dc.subject.meshNeoplasmsen_US
dc.subject.meshPrimary immunodeficiency diseasesen_US
dc.subject.meshTurkeyen_US
dc.subject.meshYoung adulten_US
dc.subject.scopusAtaxia Telangiectasia; Mutation; ATM Proteinen_US
dc.subject.wosAllergyen_US
dc.subject.wosImmunologyen_US
dc.subject.wosPediatricsen_US
dc.titleThe evaluation of malignancies in Turkish primary immunodeficiency patients; A multicenter studyen_US
dc.typeArticle
dc.wos.quartileQ1en_US

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