Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study
Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study
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DOI：
10.1182/blood-2004-09-3426
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发表时间：
2005-04-01
期刊：
影响因子：
20.3
通讯作者：
Barbui, T
中科院分区：
文献类型：
--
作者：
Finazzi, G;Caruso, V;Barbui, T
进展为急性髓系白血病/骨髓增生异常综合征 (AML/MDS) 是真性红细胞增多症 (PV) 的一种可能演变，但某些患者发生这种并发症的自然风险是否增加以及药物细胞减灭术对疾病自然病程的影响有多大仍不确定。欧洲低剂量阿司匹林治疗真性红细胞增多症合作 (ECLAP) 前瞻性项目纳入了 1638 名真性红细胞增多症患者。 22 名患者在研究招募后中位 2.5 年以及诊断 PV 后中位 8.4 年后被诊断为 AML/MDS。使用不同的多变量分析模型评估与 AML/MDS 进展相关的变量。年龄较大被证实是主要的独立危险因素（风险比 [HR]，4.30；95% 置信区间 [95% CI]，1.16-15.94；P =.0294），而总病程未能达到统计学显着性（10 年以上：HR，1.91；95% CI，0.64-5.69；P =.2466）。与相比，暴露于 P32、白消安和哌泊溴曼（HR，5.46；95% CI，1.84-16.25；P =.0023），但不单独接触羟基脲（HU）（HR，0.86；95% CI，0.26-2.88；P =.8021），在产生进展为 AML/MDS 的过高风险方面具有独立作用。静脉切开术或干扰素治疗。 (c) 2005 年，美国血液学会。
Progression to acute myeloid leukemia/myelodysplastic syndromes (AML/MDS) is a possible evolution of polycythemia vera (PV), but whether some patients are at increased natural risk for this complication and how much the contribution of pharmacologic cytoreduction can affect the natural course of the disease remain uncertain. The European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) prospective project included 1638 patients with PV. AML/MDS was diagnosed in 22 patients after a median of 2.5 years from recruitment in the study and a median of 8.4 years from the diagnosis of PV. Variables associated with progression to AML/MDS were assessed using different models of multivariate analysis. Older age was confirmed as the main independent risk factor (hazard ratio [HR], 4.30; 95% confidence interval [95% CI], 1.16-15.94; P =.0294), whereas overall disease duration failed to reach statistical significance (more than 10 years: HR, 1.91; 95% CI, 0.64-5.69; P =.2466). Exposure to P32, busulphan, and pipobroman (HR, 5.46; 95% CI, 1.84-16.25; P =.0023), but not to hydroxyurea (HU) alone (HR, 0.86; 95% CI, 0.26-2.88; P =.8021), had an independent role in producing an excess risk for progression to AML/MDS compared with treatment with phlebotomy or interferon. (c) 2005 by The American Society of Hematology.