肺高血压的特征和预后影响在患有骨髓扩散性瘤的患者中
Orly Leiva1, Steven Soo2, Olivia Liu3
1Section of Cardiology - Heart Failure, Department of Medicine, University of Chicago, Chicago, IL; Division of Cardiology, Department of Medicine, New York University Grossman School of Medicine. Orly.leiva@uchicagomedicine.org.
骨髓扩散性瘤 (MPN) 中的肺高血压 (PH) 与疾病进展和心血管事件的风险更高有关. 心声学可以帮助确定这些风险在患有基本血小板血,真多细胞血和骨髓纤维化.
科学领域:
- 血液学 血液学 血液学
- 心脏病学 心脏病学
- 在瘤学瘤学.
背景情况:
- 肺高血压 (PH) 是费城阴性髓增殖性新生瘤 (MPN) 的已知并发症,包括精髓血栓瘤 (ET),真多细胞瘤 (PV) 和骨髓纤维化 (MF).
- 在MPN患者中PH的预后影响仍未得到充分研究,因此需要进一步调查其与疾病进展和不良结果的关联.
研究的目的:
- 评估通过心声学识别的PH的预后意义,对MPN患者中转化为二次性髓纤维化 (MF) 或急性白血病的风险.
- 评估PH与主要不良心血管事件 (MACE) 和所有原因死亡率的关联.
主要方法:
- 一项多中心,回顾性队列研究,涉及MPN患者,在2010年至2023年期间记录了至少一个心声回声图.
- PH被定义为肺动脉静脉压 (PASP) ≥40 mmHg.
- 多变量细灰竞争风险回归模型被用于估计血液学进展和MACE的次危险比率 (SHR).
主要成果:
- 在555名MPN患者中 (42.7%的PV,41.1%的ET,16.2%的MF),有195名 (35.1%) 患有PH. 在51.2个月的中位随访期间,PH与二次MF进展 (aSHR 2.40),白血病进展 (aSHR 3.06) 和MACE (aSHR 1.59) 的风险显著增加有关.
- 没有发现PH和全因死亡 (aHR 1.48) 之间有显著的关联.
- 在PH患者中,缺少左心病 (LHD) 与ET/PV中二次MF进展和MF中白血病进展的风险更高相关.
结论:
- 通过心声学识别的PH是MPN患者血液进展 (二次MF和白血病) 和MACE的重要风险因素.
- 在PH患者中缺乏LHD可能表明特定血液变化的风险特别高.
- 需要进行前性研究来证实这些发现,并探索心声回声学对MPN预后的有用性.
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