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Malignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat

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dc.contributor.authorKOÇ, AHMET
dc.contributor.authorsKiykim, Ayca; Eker, Nursah; Surekli, Ozlem; Nain, Ercan; Kasap, Nurhan; Akturk, Hacer; Dogru, Omer; Canbolat, Aylin; Somer, Ayper; Koc, Ahmet; Tokuc, Gulnur; Bozkurt, Suheyla; Turkoz, Kemal; Karakoc-Aydiner, Elif; Ozen, Ahmet; Baris, Safa
dc.date.accessioned2022-03-12T22:39:18Z
dc.date.available2022-03-12T22:39:18Z
dc.date.issued2020
dc.description.abstractBackground Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. Methods Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. Results The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% +/- 12.1%. The OS for lymphoma was 62.2% +/- 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). Conclusion In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.
dc.identifier.doi10.1002/pbc.28091
dc.identifier.eissn1545-5017
dc.identifier.issn1545-5009
dc.identifier.pubmed31736244
dc.identifier.urihttps://hdl.handle.net/11424/235800
dc.identifier.wosWOS:000496750500001
dc.language.isoeng
dc.publisherWILEY
dc.relation.ispartofPEDIATRIC BLOOD & CANCER
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectcancer
dc.subjectlymphoid proliferation
dc.subjectlymphoma
dc.subjectmalignancy
dc.subjectprimary immune deficiency
dc.subjectprognosis
dc.subjectIMMUNODEFICIENCY SYNDROMES
dc.subjectIMMUNOLOGICAL PHENOTYPE
dc.subjectCANCER
dc.subjectCHILDREN
dc.subjectSECONDARY
dc.subjectMUTATION
dc.subjectPATHWAY
dc.subjectPATIENT
dc.subjectPIK3R1
dc.titleMalignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat
dc.typearticle
dspace.entity.typePublication
local.avesis.ida80513ee-f168-4778-b0ae-c388138568bf
local.import.packageSS17
local.indexed.atWOS
local.indexed.atSCOPUS
local.indexed.atPUBMED
local.journal.articlenumbere28091
local.journal.numberofpages8
local.journal.quartileQ2
oaire.citation.issue2
oaire.citation.titlePEDIATRIC BLOOD & CANCER
oaire.citation.volume67
relation.isAuthorOfPublication41bfa1f1-f3ed-4d4b-9e02-902fd20bc830
relation.isAuthorOfPublication.latestForDiscovery41bfa1f1-f3ed-4d4b-9e02-902fd20bc830

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