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

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dc.contributor.authorCekic, Sukru
dc.contributor.authorMetin, Ayse
dc.contributor.authorAytekin, Caner
dc.contributor.authorKaraca, Neslihan Edeer
dc.contributor.authorBaris, Safa
dc.contributor.authorKarali, Yasin
dc.contributor.authorKilic, Sara Sebnem
dc.date.accessioned2020-12-01T12:02:02Z
dc.date.available2020-12-01T12:02:02Z
dc.date.issued2020
dc.departmentEge Üniversitesien_US
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.doi10.1111/pai.13231en_US
dc.identifier.endpage536en_US
dc.identifier.issn0905-6157
dc.identifier.issn1399-3038
dc.identifier.issue5en_US
dc.identifier.pmid32060950en_US
dc.identifier.scopus2-s2.0-85081679990en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage528en_US
dc.identifier.urihttps://doi.org/10.1111/pai.13231
dc.identifier.urihttps://hdl.handle.net/11454/62578
dc.identifier.volume31en_US
dc.identifier.wosWOS:000550050500001en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.ispartofPediatric Allergy and Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_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.titleThe evaluation of malignancies in Turkish primary immunodeficiency patients; a multicenter studyen_US
dc.typeArticleen_US

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