Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients

Basit Öğe Kaydı
dc.contributor.authorTufekci, Ozlem
dc.contributor.authorKocak, Ulker
dc.contributor.authorKaya, Zuhre
dc.contributor.authorYenicesu, Idil
dc.contributor.authorAlbayrak, Canan
dc.contributor.authorAlbayrak, Davut
dc.contributor.authorBengoa, Sebnem Yilmaz
dc.contributor.authorPatiroglu, Turkan
dc.contributor.authorKarakukcu, Musa
dc.contributor.authorUnal, Ekrem
dc.contributor.authorInce, Elif Unal
dc.contributor.authorIleri, Talia
dc.contributor.authorErtem, Mehmet
dc.contributor.authorCelkan, Tiraje
dc.contributor.authorOzdemir, Gul Nihal
dc.contributor.authorSarper, Nazan
dc.contributor.authorKacar, Dilek
dc.contributor.authorYarali, Nese
dc.contributor.authorOzbek, Namik Yasar
dc.contributor.authorKupesiz, Alphan
dc.contributor.authorKarapinar, Tuba
dc.contributor.authorVergin, Canan
dc.contributor.authorCaliskan, Umran
dc.contributor.authorTokgoz, Huseyin
dc.contributor.authorEvim, Melike Sezgin
dc.contributor.authorBaytan, Birol
dc.contributor.authorGunes, Adalet Meral
dc.contributor.authorKarapinar, Deniz Yilmaz
dc.contributor.authorKaraman, Serap
dc.contributor.authorUygun, Vedat
dc.contributor.authorKarasu, Gulsun
dc.contributor.authorYesilipek, Mehmet Akif
dc.contributor.authorKoc, Ahmet
dc.contributor.authorErduran, Erol
dc.contributor.authorAtabay, Berna
dc.contributor.authorOniz, Haldun
dc.contributor.authorOren, Hale
dc.date.accessioned2019-10-27T10:46:31Z
dc.date.available2019-10-27T10:46:31Z
dc.date.issued2018
dc.departmentEge Üniversitesien_US
dc.description.abstractObjective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x10(9)/L, 5.4x10(9)/L, and 58.3x10(9)/L, respectively. Monosomy 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 30 +/- 17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x10(9)/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results are not satisfactory and it is necessary to put more effort into this issue in Turkey.en_US
dc.identifier.doi10.4274/tjh.2017.0021en_US
dc.identifier.endpage34en_US
dc.identifier.issn1300-7777
dc.identifier.issn1308-5263
dc.identifier.issue1en_US
dc.identifier.pmid28179213en_US
dc.identifier.startpage27en_US
dc.identifier.urihttps://doi.org/10.4274/tjh.2017.0021
dc.identifier.urihttps://hdl.handle.net/11454/31308
dc.identifier.volume35en_US
dc.identifier.wosWOS:000426572200004en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherGalenos Yayinciliken_US
dc.relation.ispartofTurkish Journal of Hematologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectHematopoietic stem cell transplantationen_US
dc.subjectJuvenile myelomonocytic leukemiaen_US
dc.subjectTurkeyen_US
dc.titleJuvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patientsen_US
dc.typeArticleen_US

Dosyalar

Koleksiyon

WoS İndeksli Yayınlar Koleksiyonu
PubMed İndeksli Yayın Koleksiyonu