由于数据不一致性,很难对该疾病进行具体的长期展望。尽管该病以无痛症状的形式存在,并且常常多年不会打扰患者近乎正常的生活,但是白血病、耗竭期、PV(后真性红细胞增多症骨髓纤维化)和严重血栓的风险一直存在。[53]Beutler E. Polcythemia. In: Beutler E, Lichtman MA, Coller BS, et al, eds. Williams hematology. 6th ed. New York: McGraw-Hill; 2001:689-701.一项纳入 1545 名 PV 患者的国际研究根据年龄、白细胞计数和静脉血栓形成病史确立了风险评分:[10]Tefferi A, Rumi E, Finazzi G, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27:1874-1881.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3768558/http://www.ncbi.nlm.nih.gov/pubmed/23739289?tool=bestpractice.com
低(中位生存期位 27.8 年)
中(中位生存期为 18.9 年)
高(中位生存期为 10.9 年)。
在该模型中,年龄≥67 周岁得 5 分,年龄为 57-66 周岁得 2 分,白细胞计数≥15 x 10^9/L(15 x 10^3/μL 或 15,000/μL)和存在静脉血栓形成分别得 1 分。
整个研究群组的中位生存期预计为 18.9 年,但当分析被局限于有着最成熟生存期数据(对 44% 的患者的随访一直持续到其死亡为止)的中心 (n = 337) 时,中位生存期仅为 14.1 年。[10]Tefferi A, Rumi E, Finazzi G, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27:1874-1881.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3768558/http://www.ncbi.nlm.nih.gov/pubmed/23739289?tool=bestpractice.com在各种大型研究中,PV 患者的最常见死因是心血管并发症[11]Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23:2224-2232.http://jco.ascopubs.org/content/23/10/2224.fullhttp://www.ncbi.nlm.nih.gov/pubmed/15710945?tool=bestpractice.com[96]Hultcrantz M, Wilkes SR, Kristinsson SY, et al. Risk and cause of death in patients diagnosed with myeloproliferative neoplasms in Sweden between 1973 and 2005: a population-based study. J Clin Oncol. 2015;33:2288-2295.http://jco.ascopubs.org/content/33/20/2288.longhttp://www.ncbi.nlm.nih.gov/pubmed/26033810?tool=bestpractice.com和转化为急性髓细胞性白血病。[11]Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23:2224-2232.http://jco.ascopubs.org/content/23/10/2224.fullhttp://www.ncbi.nlm.nih.gov/pubmed/15710945?tool=bestpractice.com[77]Finazzi G, Caruso V, Marchioli R, et al. Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study. Blood. 2005;105:2664-2670.http://bloodjournal.hematologylibrary.org/cgi/content/full/105/7/2664http://www.ncbi.nlm.nih.gov/pubmed/15585653?tool=bestpractice.com[78]Kiladjian JJ, Chevret S, Dosquet C, et al. Treatment of polycythemia vera with hydroxyurea and pipobroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011;29:3907-3913.http://jco.ascopubs.org/content/29/29/3907.longhttp://www.ncbi.nlm.nih.gov/pubmed/21911721?tool=bestpractice.com