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

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dc.contributor.authorÖZEN, AHMET OĞUZHAN
dc.contributor.authorsCekic, Sukru; Metin, Ayse; Aytekin, Caner; Karaca, Neslihan Edeer; Baris, Safa; Karali, Yasin; Kiykim, Ayca; Aydiner, Elif Karakoc; Ozen, Ahmet; Aslan, Torehan; Sevinir, Betul; Aksu, Guzide; Kutukculer, Necil; Kilic, Sara Sebnem
dc.date.accessioned2022-03-12T22:54:51Z
dc.date.available2022-03-12T22:54:51Z
dc.date.issued2020
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.
dc.identifier.doi10.1111/pai.13231
dc.identifier.eissn1399-3038
dc.identifier.issn0905-6157
dc.identifier.pubmed32060950
dc.identifier.urihttps://hdl.handle.net/11424/236556
dc.identifier.wosWOS:000550050500001
dc.language.isoeng
dc.publisherWILEY
dc.relation.ispartofPEDIATRIC ALLERGY AND IMMUNOLOGY
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectataxia-telangiectasia
dc.subjectcancer
dc.subjectDOCK8 deficiency
dc.subjectlymphoma
dc.subjectmalignancy
dc.subjectnon-Hodgkin lymphoma
dc.subjectprimary immunodeficiency
dc.subjectCANCER INCIDENCE
dc.subjectATAXIA-TELANGIECTASIA
dc.subjectLYMPHOMA
dc.subjectDISEASES
dc.subjectPATHOPHYSIOLOGY
dc.subjectSECONDARY
dc.subjectCHILDREN
dc.subjectGENE
dc.titleThe evaluation of malignancies in Turkish primary immunodeficiency patients; a multicenter study
dc.typearticle
dspace.entity.typePublication
local.avesis.iddd67c755-be09-48ec-a24c-d7268f97933d
local.import.packageSS17
local.indexed.atWOS
local.indexed.atSCOPUS
local.indexed.atPUBMED
local.journal.numberofpages9
local.journal.quartileQ1
oaire.citation.endPage536
oaire.citation.issue5
oaire.citation.startPage528
oaire.citation.titlePEDIATRIC ALLERGY AND IMMUNOLOGY
oaire.citation.volume31
relation.isAuthorOfPublication3e9c297b-e636-4836-8f61-dc9c8b7c29cf
relation.isAuthorOfPublication.latestForDiscovery3e9c297b-e636-4836-8f61-dc9c8b7c29cf

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