Soyer Nur, Haznedaroğlu İbrahim C, Cömert Melda, Çekdemir Demet, Yılmaz Mehmet, Ünal Ali, Çağlıyan Gülsüm, Bilgir Oktay, İlhan Osman, Özdemirkıran Füsun, Kaya Emin, Şahin Fahri, Vural Filiz, Saydam Güray
Ege University Faculty of Medicine, Department of Hematology, İzmir, Turkey Phone: +90 232 390 42 87 E-mail:
Turk J Haematol. 2017 Mar 1;34(1):27-33. doi: 10.4274/tjh.2016.0005. Epub 2016 Apr 18.
Chronic myeloproliferative neoplasms (CMPNs) that include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are Philadelphia-negative malignancies characterized by a clonal proliferation of one or several lineages. The aim of this report was to determine the demographic features, disease characteristics, treatment strategies, and survival rates of patients with CMPNs in Turkey.
Across all of Turkey, 9 centers were enrolled in the study. We retrospectively evaluated 708 CMPN patients' results including 390 with ET, 213 with PV, and 105 with PMF.
The JAK2V617F mutation was found positive in 86% of patients with PV, in 51.5% of patients with ET, and in 50.4% of patients with PMF. Thrombosis and bleeding at diagnosis occurred in 20.6% and 7.5% of PV patients, 15.1% and 9% of ET patients, and 9.5% and 10.4% of PMF patients, respectively. Six hundred and eight patients (85.9%) received cytoreductive therapy. The most commonly used drug was hydroxyurea (89.6%). Leukemic and fibrotic transformations occurred at rates of 0.6% and 13.2%. The estimated overall survival in PV, ET, and PMF patients was 89.7%, 85%, and 82.5% at 10 years, respectively. There were no significant differences between survival in ET, PV, and PMF patients at 10 years.
Our patients' results are generally compatible with the literature findings, except for the relatively high survival rate in PMF patients. Hydroxyurea was the most commonly used cytoreductive therapy. Our study reflects the demographic features, patient characteristics, treatments, and survival rates of Turkish CMPN patients.
慢性骨髓增殖性肿瘤（CMPN）包括真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（PMF），是一类费城染色体阴性的恶性肿瘤，其特征为一个或多个谱系的克隆性增殖。本报告旨在确定土耳其CMPN患者的人口统计学特征、疾病特征、治疗策略和生存率。
在土耳其全国范围内，9个中心参与了本研究。我们回顾性评估了708例CMPN患者的结果，其中包括390例ET患者、213例PV患者和105例PMF患者。
JAK2V617F突变在86%的PV患者、51.5%的ET患者和50.4%的PMF患者中呈阳性。诊断时血栓形成和出血分别发生在20.6%的PV患者、15.1%的ET患者和9.5%的PMF患者中，以及7.5%的PV患者、9%的ET患者和10.4%的PMF患者中。608例患者（85.9%）接受了减细胞治疗。最常用的药物是羟基脲（89.6%）。白血病转化和纤维化转化的发生率分别为0.6%和13.2%。PV、ET和PMF患者10年的估计总生存率分别为89.7%、85%和82.5%。ET、PV和PMF患者10年的生存率之间无显著差异。
除PMF患者的生存率相对较高外，我们患者的结果总体上与文献报道相符。羟基脲是最常用的减细胞治疗药物。我们的研究反映了土耳其CMPN患者的人口统计学特征、患者特征、治疗方法和生存率。
