骨髓增殖性肿瘤患者的肺动脉高压：一项包含 183 例患者的大型队列研究。
Pulmonary hypertension in patients with myeloproliferative neoplasms: A large cohort of 183 patients.
机构信息
Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France; Aix-Marseille University, INSERM, UMR1090 TAGC, Marseille F_13288, France.
Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France; General Medicine Department, Aix-Marseille University, France.
出版信息
Eur J Intern Med. 2019 Oct;68:71-75. doi: 10.1016/j.ejim.2019.08.004. Epub 2019 Aug 14.
BACKGROUND
Chronic myeloproliferative neoplasms (MPN) are recognized as a cause of pulmonary hypertension (pH). We ought to describe the prevalence and characteristics of PH in a cohort of MPN who were screened using transthoracic echocardiography (TTE).
METHODS
One hundred eighty-three newly diagnosed consecutive MPN patients were prospectively evaluated using TTE to detect PH.
RESULTS
Two patients were diagnosed with chronic eosinophilic leukemia, two patients had post-essential thrombocythemia (ET) myelofibrosis (MF), two patients had post-polycythemia vera (PV) MF, 11 patients had primary myelofibrosis (PMF), 28 patients had chronic myeloid leukemia (CML), 51 patients had PV, and 87 patients had ET. TTE was used to determine PH, and PH was suspected in 16 of 183 patients as follows: four with PV, seven with ET, two with PMF, and three with CML. Two patients with ET were excluded because of global cardiac failure. Three patients underwent right heart catheterization to confirm PH. The 14 (7.7%) patients with PH had no cardiac or lung disease that directly involved MPN in PH development.
CONCLUSION
In this large cohort of 183 MPN patients, TTE was used to diagnose PH, and 14 patients (7.7%) developed PH. This prevalence was lower than expected based on previously reported data, but it remains higher than in the general population.
背景
慢性骨髓增生性肿瘤（MPN）被认为是肺动脉高压（PH）的病因之一。我们应该描述一组经胸超声心动图（TTE）筛查的 MPN 患者中 PH 的患病率和特征。
方法
183 例新诊断的连续 MPN 患者前瞻性地接受 TTE 评估，以检测 PH。
结果
两名患者被诊断为慢性嗜酸性白血病，两名患者为原发性血小板增多症（ET）后骨髓纤维化（MF），两名患者为真性红细胞增多症（PV）后 MF，11 名患者为原发性骨髓纤维化（PMF），28 名患者为慢性髓性白血病（CML），51 名患者为 PV，87 名患者为 ET。TTE 用于确定 PH，183 例患者中有 16 例疑似 PH，如下所示：4 例 PV、7 例 ET、2 例 PMF 和 3 例 CML。由于全心衰竭，ET 中有 2 例患者被排除在外。有 3 例患者进行了右心导管检查以确认 PH。在 14 例（7.7%）患有 PH 的患者中，没有直接涉及 PH 发展的 MPN 的心脏或肺部疾病。
结论
在这项 183 例 MPN 患者的大型队列研究中，TTE 用于诊断 PH，14 例（7.7%）患者出现 PH。这一患病率低于先前报告的数据预期，但仍高于一般人群。
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