Leiva Orly, Ren Siyang, Neuberg Donna, Bhatt Ankeet, Jenkins Andrew, Rosovsky Rachel, Karp Leaf Rebecca, Goodarzi Katayoon, Hobbs Gabriela
Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
Division of Cardiovascular Medicine, Department of Medicine, New York University Langone Health, New York, NY, USA.
Int J Hematol. 2023 Jan;117(1):90-99. doi: 10.1007/s12185-022-03454-1. Epub 2022 Oct 2.
Cardiovascular events and hematologic progression to myelofibrosis or leukemia are leading causes of morbidity and mortality among patients with myeloproliferative neoplasms (MPN). Pulmonary hypertension (PH) is also associated with MPN and cardiovascular disease (CVD), though its prognostic significance in MPN is not well characterized. Our primary objective was to investigate the effect of PH, defined as right-ventricular systolic pressure (RVSP) ≥ 50 mmHg on echocardiogram or mean pulmonary artery pressure (mPAP) ≥ 20 on right heart catheterization, on cardiovascular and all-cause mortality and hematologic progression in patients with MPN and CVD (atrial fibrillation, heart failure hospitalization, and myocardial infarction after MPN diagnosis). Of the 197 patients included (86 ET, 80 PV, 31 PMF), 92 (47%) had PH and 98 (50%) were male. All-cause mortality (58 vs 37%, p = 0.004), cardiovascular death (35 vs 9%, p < 0.0001), and hematologic progression (23 vs 11%, p = 0.037) occurred more frequently in patients with PH. Multivariable competing-risk and proportional hazards regression showed that PH was associated with increased risk of all-cause death (adjusted hazard ratio [HR], 1.80, 95% CI 1.10-2.93), CV death (adjusted subdistribution HR 3.71, 95% CI 1.58-8.73), and hematologic progression (adjusted subdistribution HR 1.99, 95% CI 1.21-3.27).
心血管事件以及向骨髓纤维化或白血病的血液学进展是骨髓增殖性肿瘤（MPN）患者发病和死亡的主要原因。肺动脉高压（PH）也与MPN和心血管疾病（CVD）相关，尽管其在MPN中的预后意义尚未得到充分阐明。我们的主要目的是研究PH（定义为超声心动图显示右心室收缩压（RVSP）≥50 mmHg或右心导管检查显示平均肺动脉压（mPAP）≥20 mmHg）对MPN合并CVD（心房颤动、心力衰竭住院以及MPN诊断后心肌梗死）患者心血管和全因死亡率以及血液学进展的影响。在纳入的197例患者中（86例真性红细胞增多症、80例原发性血小板增多症、31例原发性骨髓纤维化），92例（47%）有PH，98例（50%）为男性。PH患者的全因死亡率（58%对37%，p = 0.004）、心血管死亡（35%对9%，p < 0.0001）和血液学进展（23%对11%，p = 0.037）更常见。多变量竞争风险和比例风险回归显示，PH与全因死亡风险增加相关（调整后风险比[HR]，1.80，95%置信区间1.10 - 2.93）、心血管死亡（调整后亚分布HR 3.71，95%置信区间1.58 - 8.73）和血液学进展（调整后亚分布HR 1.99，95%置信区间1.21 - 3.27）。
