Malignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience

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dc.contributor.authorAydogmus, Cigdem
dc.contributor.authorTurkyilmaz Ucar, Ozge
dc.contributor.authorKaplan Sarikavak, Sibel
dc.contributor.authorCipe, Funda
dc.contributor.authorUlas, Selami
dc.contributor.authorTuran, Isilay
dc.contributor.authorNaiboglu, Sezin
dc.contributor.authorYildirim, Ilke
dc.contributor.authorKalay, Gulsah
dc.contributor.authorKapci, Nermin
dc.contributor.authorAl, Serdar
dc.contributor.authorGokmirza Ozdemir, Pinar
dc.contributor.authorCeliksoy, Mehmet Halil
dc.date.accessioned2024-02-20T11:57:00Z
dc.date.available2024-02-20T11:57:00Z
dc.date.issued2024en_US
dc.departmentFakülteler, Tıp Fakültesien_US
dc.description.abstractPrimary immune deficiencies (PIDs) are rare genetic disorders characterized by impaired immune function, leading to frequent infections and immune dysregulation. Studies have shown that individuals with PID are at an increased risk of developing malignancies and lymphoproliferative disorders compared with the general population. In this single-center study, we aimed to analyze the occurrence of malignancies and lymphoproliferations in children diagnosed with PID. We retrospectively analyzed the medical records of 550 pediatric patients diagnosed with PIDs at our center. Among them, 17 (3,0%) patients were identified with malignancy and/or benign lymphoproliferation. Eight of the 17 patients (47.0%) had immune dysregulatory diseases, whereas ataxia-telangiectasia was the second most common PID associated with malignancy and/or benign lymphoproliferation (n = 5, 29.4%). Lymphoma was the predominant malignancy (n = 11, 64.7%), and Epstein-Barr virus was identified as the most common viral agent associated with malignancy and/or benign lymphoproliferation in patients with PID (n = 8, 47.0%). Our study highlights the association between PID and malignancies/lymphoproliferations, with immune dysregulation syndromes being the most common subclass associated with malignancies/lymphoproliferations. Early diagnosis, multidisciplinary management, and regular surveillance are crucial in improving patient outcomes and saving lives.en_US
dc.identifier.citationAydogmus, C., Ucar, O. T., Sarikavak, S. K., Cipe, F., Ulas, S., Turan, I., Naiboglu, S., Turan, I., Kalay, G., Yildirim, I., Kapci, N., Al, S., Gokmirza Ozdemir, P., Celiksoy, M. H. (2024). Malignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience. Journal of Pediatric Hematology/Oncology, 46(2), e169-e173. 10.1097/MPH.0000000000002817en_US
dc.identifier.endpagee173en_US
dc.identifier.issn1077-4114
dc.identifier.issn1536-3678
dc.identifier.issue2en_US
dc.identifier.scopus2-s2.0-85186265315
dc.identifier.scopusqualityQ3
dc.identifier.startpagee169en_US
dc.identifier.urihttps://hdl.handle.net/20.500.12939/4592
dc.identifier.volume46en_US
dc.identifier.wosWOS:001177433500013
dc.identifier.wosqualityQ3
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorCipe, Funda
dc.language.isoen
dc.relation.ispartofJournal of Pediatric Hematology/Oncology
dc.relation.isversionof10.1097/MPH.0000000000002817en_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectPrimary immune deficiencies (PIDs)en_US
dc.subjectGenetic disordersen_US
dc.subjectİmmune functionen_US
dc.titleMalignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience
dc.typeArticle

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