FROM Research Foundation, Papa Giovanni XXIII Hospital, Bergamo, Italy.
Department of Medicine, Weill Cornell Medicine, New York, NY, USA.
Eur J Haematol. 2021 Feb;106(2):250-259. doi: 10.1111/ejh.13543. Epub 2020 Nov 22.
Pulmonary hypertension (PH) is commonly reported in Philadelphia-chromosome negative myeloproliferative neoplasms (MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). PH may be diagnosed directly by right heart catheterization (RHC) or estimated by transthoracic echocardiography (TTE). Survival is shortened by PH but despite the potential significance of PH to management and prognosis of MPN, estimates of its prevalence in MPNs vary and risk factors for the condition are poorly established. We performed a systematic review and meta-analysis of available studies to fill this void.
We searched EMBASE, MEDLINE, and Clinicaltrials.gov for the terms "pulmonary hypertension," "myeloproliferative disorders," "polycythemia vera," "essential thrombocythemia," and "myelofibrosis." We restricted analysis to the 1999-2019 window to improve uniformity of MPN diagnostic criteria. We retrieved 221 records and, after abstract and full-text screening, identified 17 papers meeting criteria for inclusion in our meta-analysis. A modified Newcastle-Ottawa scale was used to assess quality.
Results for 935 patients were available, 309 of these having PH (33%). Using logistic mixed-effect regression, we found that diagnosis mode (RHC vs TTE) and MPN duration influenced PH prevalence. Studies employing predominantly TTE yielded prevalence estimates ~5-fold higher than those using RHC (35% vs 7.2%). We identified MF and duration of MPN as significant risk factors for development of PH.
Prevalence of PH in MPNs is poorly understood with estimates ranging from 3.8% to 58%. Patients with MF and longer duration of disease seem at particularly high risk and should be carefully monitored for PH.
肺高血压（PH）在费城染色体阴性骨髓增殖性肿瘤（MPN）中较为常见，包括真性红细胞增多症（PV）、原发性血小板增多症（ET）和骨髓纤维化（MF）。PH 可通过右心导管检查（RHC）直接诊断，也可通过经胸超声心动图（TTE）估计。PH 会缩短生存期，但尽管 PH 对 MPN 的治疗和预后有潜在意义，但 MPN 中 PH 的患病率估计值存在差异，其发病风险因素也尚未明确。我们进行了系统评价和荟萃分析，以填补这一空白。
我们在 EMBASE、MEDLINE 和 Clinicaltrials.gov 中检索了“肺高血压”、“骨髓增殖性疾病”、“真性红细胞增多症”、“原发性血小板增多症”和“骨髓纤维化”等术语。我们将分析限制在 1999 年至 2019 年的窗口期，以提高 MPN 诊断标准的一致性。我们检索到 221 条记录，经过摘要和全文筛选，确定了 17 篇符合纳入荟萃分析标准的论文。我们使用改良的 Newcastle-Ottawa 量表评估质量。
有 935 名患者的结果可用，其中 309 名患有 PH（33%）。使用逻辑混合效应回归，我们发现诊断方式（RHC 与 TTE）和 MPN 持续时间影响 PH 的患病率。主要采用 TTE 的研究得出的患病率估计值比采用 RHC 的研究高出约 5 倍（35%比 7.2%）。我们确定 MF 和 MPN 持续时间是 PH 发生的重要危险因素。
MPN 中 PH 的患病率尚不清楚，估计值范围为 3.8%至 58%。MF 患者和疾病持续时间较长的患者似乎风险特别高，应密切监测 PH。
