单中心906例真性红细胞增多症患者生存预后因素分析

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). CONCLUSION: 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.