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按照世界卫生组织标准严格定义的真性红细胞增多症患者的表现模式和血栓形成结局,并按诊断的日历时间进行分层。

Patterns of presentation and thrombosis outcome in patients with polycythemia vera strictly defined by WHO-criteria and stratified by calendar period of diagnosis.

机构信息

Research Foundation Papa Giovanni XXIII Hospital, Bergamo, Italy.

出版信息

Am J Hematol. 2015 May;90(5):434-7. doi: 10.1002/ajh.23970. Epub 2015 Feb 27.

Abstract

Most studies in polycythemia vera (PV) include patients with both remote and most recent diagnostic periods and are therefore vulnerable to inaccurate interpretation of time-dependent data. We addressed the particular issue by analyzing presenting characteristics and outcome data among 1,545 patients with WHO-defined PV stratified by a diagnosis period of before or after 2005, which coincides with the first description of JAK2V617F as the molecular marker of PV. Patients diagnosed after 2005 displayed lower hemoglobin values (P < 0.0001) and older age (P = 0.007) at diagnosis; we suggest ease of diagnosis offered by a molecular marker enabled earlier diagnosis and broader application across older age groups that is further enhanced by recent trends in increased attention and health monitoring for the elderly. Post-2005 diagnosed patients were also more or less likely to receive aspirin and cytoreductive therapy, respectively, and, despite their older age distribution, displayed significantly lower risk of thrombosis in high risk disease. Regardless of the contributing factors to the latter phenomenon, our observations underscore the need to reassess current demographics and frequencies of thrombosis in clinical trial designs including thrombosis prevention in PV.

摘要

大多数真性红细胞增多症 (PV) 的研究都包括了最近和很久以前诊断的患者,因此这些研究结果容易对依赖时间的数据产生错误的解读。我们通过分析 1545 例经世界卫生组织 (WHO) 诊断为 PV 的患者的特征和结局数据,解决了这个问题,这些患者被分为诊断时间在 2005 年之前或之后的两个组,这个时间点与 JAK2V617F 作为 PV 的分子标志物的首次描述相符。2005 年后诊断的患者的血红蛋白值较低 (P < 0.0001),且年龄较大 (P = 0.007);我们认为,分子标志物的易于诊断使患者更早地被诊断出来,诊断范围更广,并且更易被应用于年龄更大的人群,最近老年人健康监测的增加趋势进一步促进了这一情况。2005 年后诊断的患者更有可能分别接受阿司匹林和细胞毒性治疗,尽管他们的年龄分布较大,但在高危疾病中,血栓形成的风险显著降低。无论导致后一种现象的因素是什么,我们的观察结果都强调了在临床试验设计中,包括在 PV 中预防血栓形成的临床试验设计中,重新评估当前的人口统计学和血栓形成频率的必要性。

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