真性红细胞增多症和原发性血小板增多症患者的预期寿命及生存预后因素。

Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia.

作者信息

Passamonti Francesco, Rumi Elisa, Pungolino Ester, Malabarba Lucia, Bertazzoni Paola, Valentini Marina, Orlandi Ester, Arcaini Luca, Brusamolino Ercole, Pascutto Cristiana, Cazzola Mario, Morra Enrica, Lazzarino Mario

机构信息

Division of Hematology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.

出版信息

Am J Med. 2004 Nov 15;117(10):755-61. doi: 10.1016/j.amjmed.2004.06.032.

DOI:10.1016/j.amjmed.2004.06.032

PMID:15541325

Abstract

PURPOSE

To assess life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia.

METHODS

The study sample consisted of 831 consecutive patients with polycythemia vera (n = 396; 4184 person-years of follow-up) or essential thrombocythemia (n = 435; 4304 person-years of follow-up). Mortality in each group was compared with the Italian population using the standardized mortality ratio (SMR) based on life expectancy data obtained from the Italian Institute of Statistics.

RESULTS

The 15-year survival was 65% in patients with polycythemia and 73% in those with thrombocythemia. By Cox regression analysis, the independent predictors of death were a history of thrombosis for polycythemia (hazard ratio [HR] = 2.2; P = 0.0002) and thrombocythemia (HR = 2; P = 0.01), and male sex (HR = 1.8; P = 0.03) for thrombocythemia. Mortality compared with the general population was 1.6-fold higher (P <0.001) in patients with polycythemia but was not increased in those with thrombocythemia (SMR = 1; P = 0.8).

CONCLUSION

Life expectancy of patients with polycythemia vera (especially if younger than 50 years) was reduced compared with the general population, whereas life expectancy of patients with essential thrombocythemia was not affected significantly by the disease, reflecting the more indolent nature of the proliferation. History of thrombosis was the main predictor of death in both diseases.

摘要

目的

评估真性红细胞增多症和原发性血小板增多症患者的预期寿命及生存预后因素。

方法

研究样本包括831例连续的真性红细胞增多症患者（n = 396；4184人年随访）或原发性血小板增多症患者（n = 435；4304人年随访）。根据从意大利统计局获得的预期寿命数据，使用标准化死亡率（SMR）将每组的死亡率与意大利人群进行比较。

结果

真性红细胞增多症患者的15年生存率为65%，血小板增多症患者为73%。通过Cox回归分析，死亡的独立预测因素在真性红细胞增多症中为血栓形成史（风险比[HR]=2.2；P = 0.0002），在血小板增多症中为血栓形成史（HR = 2；P = 0.01）以及男性性别（HR = 1.8；P = 0.03）。真性红细胞增多症患者与普通人群相比死亡率高1.6倍（P <0.001），但血小板增多症患者死亡率未增加（SMR = 1；P = 0.8）。