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dc.contributor.authorAkturk, Hacer
dc.contributor.authorBARIŞ, SAFA
dc.contributor.authorOzen, Ahmet
dc.contributor.authorKarakoc-Aydiner, Elif
dc.contributor.authorTurkoz, Kemal
dc.contributor.authorBOZKURT, SÜHEYLA
dc.contributor.authorTokuc, Gulnur
dc.contributor.authorKOÇ, AHMET
dc.contributor.authorCanbolat, Aylin
dc.contributor.authorDOĞRU, ÖMER
dc.contributor.authorSomer, Ayper
dc.contributor.authorKiykim, Ayca
dc.contributor.authorEKER, NURŞAH
dc.contributor.authorSÜREKLİ KARAKUŞ, ÖZLEM
dc.contributor.authorNain, Ercan
dc.contributor.authorKasap, Nurhan
dc.date.accessioned2021-03-05T11:24:26Z
dc.date.available2021-03-05T11:24:26Z
dc.identifier.citationKiykim A., EKER N., SÜREKLİ KARAKUŞ Ö., Nain E., Kasap N., Akturk H., DOĞRU Ö., Canbolat A., Somer A., KOÇ A., et al., "Malignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat", PEDIATRIC BLOOD & CANCER, 2019
dc.identifier.issn1545-5009
dc.identifier.othervv_1032021
dc.identifier.otherav_a80513ee-f168-4778-b0ae-c388138568bf
dc.identifier.urihttp://hdl.handle.net/20.500.12627/112300
dc.identifier.urihttps://doi.org/10.1002/pbc.28091
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.language.isoeng
dc.subjectÇocuk Sağlığı ve Hastalıkları
dc.subjectHEMATOLOJİ
dc.subjectPEDİATRİ
dc.subjectTıp
dc.subjectSağlık Bilimleri
dc.subjectDahili Tıp Bilimleri
dc.subjectOnkoloji
dc.subjectHematoloji
dc.subjectİç Hastalıkları
dc.subjectONKOLOJİ
dc.subjectKlinik Tıp
dc.subjectKlinik Tıp (MED)
dc.titleMalignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat
dc.typeMakale
dc.relation.journalPEDIATRIC BLOOD & CANCER
dc.contributor.departmentMarmara Üniversitesi , ,
dc.contributor.firstauthorID270389


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