Jindamai Yanika, Rattarittamrong Ekarat, Phrommintikul Arintaya, Yongsmith Lalita, Piriyakhuntorn Pokpong, Rattanathammethee Thanawat, Hantrakool Sasinee, Chai-Adisaksopha Chatree, Tantiworawit Adisak, Norasetthada Lalita
Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, 110 Inthawaroros Street, Muang, Chiang Mai, 50200, Thailand.
Int J Hematol. 2020 Nov;112(5):631-639. doi: 10.1007/s12185-020-02952-4. Epub 2020 Jul 23.
Pulmonary hypertension (PH) is emerging as a complication of myeloproliferative neoplasms (MPNs). This was a prospective study conducted at Chiang Mai University Hospital. The primary objective was to determine the prevalence of high echocardiographic probability of PH in MPNs patients. The secondary objectives were to determine risk factors of PH and the correlation between risk factors and peak tricuspid regurgitation velocity (TRV). All MPNs patients aged over 18 years, including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), were enrolled. All eligible patients underwent echocardiography for evaluation of the probability of PH in line with the 2015 European Society of Cardiology (ESC) and the European Respiratory Society (ERS) Guidelines. Sixty-six patients with a median age of 59 years were enrolled. There were 35 PV, 25 ET, and 6 PMF patients included. The prevalence of high echocardiographic probability of PH was 4.55% (2 ET and 1 PMF). Risk factors associated with high echocardiographic probability of PHT were not analyzed. There were three risk factors associated with increased TRV specifically lower hemoglobin, older age, and the presence of JAK2V617F mutation. The prevalence of high echocardiographic probability of PH in MPNs patients was 4.55%.
肺动脉高压（PH）正逐渐成为骨髓增殖性肿瘤（MPN）的一种并发症。这是一项在清迈大学医院进行的前瞻性研究。主要目的是确定MPN患者中超声心动图提示PH可能性高的患病率。次要目的是确定PH的危险因素以及危险因素与三尖瓣反流峰值速度（TRV）之间的相关性。纳入所有年龄超过18岁的MPN患者，包括真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（PMF）。所有符合条件的患者均接受超声心动图检查，以根据2015年欧洲心脏病学会（ESC）和欧洲呼吸学会（ERS）指南评估PH的可能性。共纳入66例患者，中位年龄为59岁。其中包括35例PV患者、25例ET患者和6例PMF患者。超声心动图提示PH可能性高的患病率为4.55%（2例ET患者和1例PMF患者）。未分析与超声心动图提示PHT可能性高相关的危险因素。有三个与TRV升高相关的危险因素，具体为血红蛋白水平较低、年龄较大以及存在JAK2V617F突变。MPN患者中超声心动图提示PH可能性高的患病率为4.55%。
