Objective: To explore predictors of overall survival (OS) in Chinese patients with polycythemia vera (PV) . Methods: A total of 906 consecutive newly diagnosed patients with PV seen at the Blood Diseases Hospital, Chinese Academy of Medical Sciences, from June 2007 to February 2020 were included, and their data were collected. PV was diagnosed according to 2016 World Health Organization (WHO) diagnostic definitions. OS and prognostic factors were retrospectively analyzed. Results: Among the 906 patients, 439 were male (48.5%) and 467 were female (51.5%) . The median age was 57 years (range: 18-91 years) . 31.6% (276/874) of the patients had a thrombosis history at diagnosis, and 4.6% (25/541) of the patients had abnormal cytogenetics. The median follow-up was 54 months (95% confidence interval [CI] 8-130 months) . The 5- and 10-year cumulative deaths were 5.8% (95% CI 4.8%-6.7%) and 11.1% (95% CI 9.3%-12.9%) , respectively. Univariate analysis showed that age ≥60 years, thrombosis history, white blood cells (WBC) ≥15×10(9)/L, platelet (PLT) ≥450×10(9)/L, and platelet distribution width (PDW) ≥15 fl significantly correlated with worse OS, and palpable spleen correlated with better OS. Multivariate analysis showed that age ≥60 years (HR=4.3, 95% CI 2.1-9.2, P<0.001) and PDW ≥15 fl (HR=2.1, 95% CI 1.1-4.0, P=0.023) were independent prognostic factors for worse OS. The 5-year cumulative death for patients with PDW ≥15 fl or PDW<15 fl was 8.6% (95% CI 5.9%-11.3%) or 4.4% (95% CI 3.4%-5.4%) , respectively. The 5-year cumulative death for patients defined as low-, intermediate-, and high-risk patients by international working group score system for PV (IWG-PV) were 0.8% (95 CI 0.2%-1.4%) , 4.0% (95% CI 2.7%-5.3%) , and 12% (95% CI 9.6%-14.4%) , respectively, with a significant difference among the three cohorts (P<0.05) . PDW ≥ 15 fl significantly affected OS for intermediate- and high-risk patients (HR=2.3, 95% CI 1.2-4.2, P=0.009) defined by IWG-PV score system, but not for low-risk patients (HR=3.1, 95% CI 0.2-52.0, P=0.405) . Conclusions: Age ≥60 years and PDW ≥15 fl were independent prognostic factors for worse OS in PV. IWG-PV score system effectively predicted OS for Chinese patients with PV.
目的： 探讨影响真性红细胞增多症（PV）患者总生存（OS）的预后因素。 方法： 对2007年6月至2020年2月就诊于中国医学科学院血液病医院的906例符合WHO（2016）诊断分型标准的初诊PV患者进行回顾性分析。 结果： 906例患者中，男439例（48.5%），女467例（51.5%），中位确诊年龄为57（18~91）岁。31.6%（276/874）的患者有血栓史，27.2%（232/852）的患者左肋缘下可触及脾脏，4.6%（25/541）的患者伴有克隆性染色体核型异常。中位随访时间为54（95%CI 8~130）个月，5、10年累积死亡率分别为5.8%（95% CI 4.8%~6.7%）、11.1%（95%CI 9.3%~12.9%）。单因素分析显示，年龄≥60岁、既往有血栓史、WBC≥15×10(9)/L、PLT≥450×10(9)/L、血小板分布宽度（PDW）≥15 fl是OS的危险因素，脾脏左肋缘下可触及是OS的保护因素。Cox回归多因素分析显示，年龄≥60岁（HR=4.3，95%CI 2.1~9.2，P<0.001）、PDW≥15 fl（HR=2.1，95%CI 1.1~4.0，P=0.023）是OS的独立危险因素。PDW≥15 fl、<15 fl患者5年累积死亡率分别为8.6%（95%CI 5.9%~11.3%）、4.4%（95%CI 3.4%~5.4%）。国际工作组PV预后积分系统（IWG-PV）低危、中危、高危组患者5年累积死亡率分别为0.8%（95%CI 0.2%~1.4%）、4.0%（95%CI 2.7%~5.3%）、12.0%（95%CI 9.6%~14.4%）（P<0.05）。PDW对IWG-PV中危、高危患者的OS具有显著影响（HR=2.3，95%CI 1.2~4.2，P=0.009），而对低危患者没有影响（HR=3.1，95%CI 0.2~52.0，P=0.405）。 结论： 年龄≥60岁和PDW≥15 fl是影响中国PV患者OS的独立危险因素。IWG-PV预后积分系统适用于中国PV患者生存预测。.
Keywords: Overall survival; Platelet distribution width; Polycythemia vera; Prognosis.