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294例土耳其慢性骨髓增殖性肿瘤患者的临床特征

Clinical Features of 294 Turkish Patients with Chronic Myeloproliferative Neoplasms.

作者信息

Andıç Neslihan, Ünübol Mustafa, Yağcı Eren, Akay Olga Meltem, Yavaşoğlu İrfan, Kadıköylü Vefki Gürhan, Bolaman Ali Zahit

机构信息

Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Turkey, Phone: +90 532 518 22 63, E-mail:

出版信息

Turk J Haematol. 2016 Sep 5;33(3):187-95. doi: 10.4274/tjh.2015.0041. Epub 2016 Apr 18.

Abstract

OBJECTIVE

Myeloproliferative neoplasms (MPNs) share common clonal stem cells but show significant differences in their clinical courses. The aim of this retrospective study was to evaluate thrombotic and hemorrhagic complications, JAK2 status, gastrointestinal and cardiac changes, treatment modalities, and survival in MPNs in Turkish patients.

MATERIALS AND METHODS

Medical files of 294 patients [112 essential thrombocythemia (ET), 117 polycythemia vera (PV), 46 primary myelofibrosis, and 19 unclassified MPN cases] from 2 different universities in Turkey were examined.

RESULTS

Older age, higher leukocyte count at diagnosis, and JAK2 mutation positivity were risk factors for thrombosis. Platelet count over 1000x109/L was a risk factor for hemorrhagic episodes. Hydroxyurea treatment was not related to leukemic transformation. Median follow-up time was 50 months (quartiles: 22.2-81.75) in these patients. Patients with primary myelofibrosis had the shortest survival of 137 months when compared with 179 months for ET and 231 months for PV. Leukemic transformation, thromboembolic events, age over 60 years, and anemia were found to be the factors affecting survival.

CONCLUSION

Thromboembolic complications are the most important preventable risk factors for morbidity and mortality in MPNs. Drug management in MPNs is done according to hemoglobin and platelet counts. Based on the current study population our results support the idea that leukocytosis and JAK2 positivity are more important risk factors for thrombosis than hemoglobin and platelet values.

摘要

目的

骨髓增殖性肿瘤(MPN)具有共同的克隆干细胞,但临床病程存在显著差异。这项回顾性研究的目的是评估土耳其患者MPN的血栓形成和出血并发症、JAK2状态、胃肠道和心脏变化、治疗方式及生存率。

材料与方法

检查了来自土耳其2所不同大学的294例患者的病历[112例原发性血小板增多症(ET)、117例真性红细胞增多症(PV)、46例原发性骨髓纤维化及19例未分类的MPN病例]。

结果

年龄较大、诊断时白细胞计数较高及JAK2突变阳性是血栓形成的危险因素。血小板计数超过1000×10⁹/L是出血事件的危险因素。羟基脲治疗与白血病转化无关。这些患者的中位随访时间为50个月(四分位数:22.2 - 81.75)。与ET的179个月和PV的231个月相比,原发性骨髓纤维化患者的生存期最短,为137个月。发现白血病转化、血栓栓塞事件、年龄超过60岁及贫血是影响生存的因素。

结论

血栓栓塞并发症是MPN发病和死亡最重要的可预防危险因素。MPN的药物治疗根据血红蛋白和血小板计数进行。基于当前的研究人群,我们的结果支持以下观点,即白细胞增多和JAK2阳性比血红蛋白和血小板值是更重要的血栓形成危险因素。

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Myeloproliferative neoplasms and thrombosis.骨髓增殖性肿瘤与血栓。
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