老年真性红细胞增多症患者临床特征及预后分析

Clinical feature and prognosis analysis of elderly patients with polythemia vera

目的 分析老年真性红细胞增多症（PV）患者的疾病特征及预后.方法 回顾性分析自2009年1月至2013年12月就诊于天津市第一中心医院血液科诊断为PV的患者,共140例,包括68例年龄≥60岁的老年PV患者和72例年龄＜60岁的非老年PV患者,对老年PV患者的疾病特点和临床结果进行分析,并与非老年患者进行比较.结果 老年组患者较非老年组患者具有更多的血栓病史[54.4％ （37/68）比30.6％（22/72）,P=0.004],较高的心脑血管危险因素[63.2％ （43/68）比36.1％（26/72）,P=0.001],较高的高白细胞[（13.9 ±3.8）×109/L比（7.8 ±2.2）×109/L,P=0.000）和JAK2V617F基因突变负荷[62％ （30％ ～81％）比41％ （26％ ～63％）,P=0.035].老年组患者较非老年组患者血管并发症发生率更高[54.4％（37/68）比30.6％（22/72）,P=0.004],更易向骨髓纤维化转化[11.8％ （8/68）比2.8％（2/72）,P=0.039],二者白血病转化率分别为4.4％ （3/68）和0（0/72）,但差异无统计学意义（P =0.112）.老年组患者预后不佳,较非老年组患者有更高的病死率[14.7％ （10/68）比4.2％（3/72）,P=0.032].结论 老年PV患者危险因素增多,多为高危患者,更易合并血栓及向骨髓纤维化、白血病转化.预防或延迟并发症发生是目前治疗目标,以减少疾病进展及病死率。

Objective To analyze the clinical features of elderly patients with polythemia vera （PV） Methods Statistical analyses were performed for the clinical features of 68 PV patients of age ≥ 60 years and 72 PV patients of age 〈 60 years from January 2009 to December 2013 in our hospital.Results Compared with younger patients,elderly patients with PV had higher incidences of thrombosis （54.4% （37/68） vs 30.6% （22/72）,P =0.004）,more risk factors of cardio-cerebrovascular （63.2% （43/68） vs 36.1% （26/72）,P =0.001）,higher white blood cell counts （（13.9 ± 3.8） × 109/L vs （7.8 ± 2.2） ×109/L,P =0.000） and higher JAK2 V617F allele burden（62% （30%-81%） vs 41% （26%-63%）,P =0.035）.There was higher incidences of vascular complications in elderly patients with PV （54.4% （37/ 68） vs 30.6% （22/72）,P =0.004）.Myelofibrosis transformation occurred with higher frequency in elderly patients with PV （11.8 % （8/68） vs 2.8 % （2/72）,P =0.039）.And there was no significant difference in the frequency of leukemia transformation between elderly patients and younger patients （4.4% （3/68）vs 0 （0/72）,P =0.112）.There was higher mortality rate in elderly patients with PV （14.7% （10/86） vs 4.2% （3/72）,P =0.032）.Conclusions There are more risk factors in elderly patients with PV.The elderly patients with PV has high risk to complicat with thrombosis and transformated to myelofibrosis and leukemia.Prevention or delay of these complications is currently the goal of treatment,so that it could reduce the mortality rate and disease progression.