Suolitiken Dina, Han Xue, Feng Cuicui, Wang Yini
Department of Hematology, Capital Medical University Affiliated Beijing Anzhen Hospital, Beijing, 100029, China.
Ann Hematol. 2025 Apr;104(4):2279-2285. doi: 10.1007/s00277-025-06334-w. Epub 2025 Apr 21.
Philadelphia chromosome-negative myeloproliferative neoplasms (Ph-MPNs) are a group of malignant clonal disorders originating from bone marrow hematopoietic stem cells, and pulmonary hypertension (PH) is a serious progressive disease often coexisting with Ph-MPNs, with a prevalence ranging from 5 to 50%. The aim of this study was to analyze the prevalence, clinical characteristics, and associated risk factors of PH in 130 patients with Ph-MPNs, to investigate the impact of PH on patients' prognosis, and to provide a reference for the clinical identification of adverse prognostic factors and early prevention and treatment of PH. One hundred and thirty patients with Ph-MPNs treated at Beijing Anzhen Hospital from January 1, 2020 to December 31, 2024 were included in the study. PH risk was assessed by echocardiography (ECHO), and tricuspid regurgitation velocity (TRV) > 2.8 m/s was used as the criterion for high risk of PH. General information, hematological indices, biochemical indices, gene mutations and echocardiographic data of the patients were collected and statistically analyzed. The overall prevalence of PH among the 130 patients with Ph-MPNs was 15.4%. patients with PMF had the highest prevalence of PH (72.3%), which was significantly higher than that of patients with PV (9.9%) and ET (10.4%) (P < 0.05). patients in the PH high-risk group were older, had lower hemoglobin levels, and had a higher prevalence of splenomegaly and secondary myelofibrosis. the PH high-risk group Patients had a significantly higher mortality rate than the normal risk group (20% vs. 1.81%, P = 0.0004). Multifactorial Cox regression analysis showed that advanced age (HR = 1.029, P = 0.0332) and high risk of PH (HR = 1.034, P = 0.0432) were independent risk factors for patient survival.Logistic regression analysis showed that decreased hemoglobin (OR = 0.9657, P = 0.0062), splenomegaly (OR = 5.105, P = 0.0413) and secondary myelofibrosis (OR = 7.959, P = 0.0321) were independent risk factors for high risk of PH. This study revealed the prevalence of PH, risk factors, and their prognostic implications in patients with Ph-MPNs. It is suggested that regular monitoring of changes in relevant risk factors and vigilance and prevention of PH during the treatment of patients with Ph-MPNs are clinically important to improve the prognosis of patients.
费城染色体阴性骨髓增殖性肿瘤（Ph-MPNs）是一组起源于骨髓造血干细胞的恶性克隆性疾病，而肺动脉高压（PH）是一种常与Ph-MPNs共存的严重进展性疾病，患病率在5%至50%之间。本研究的目的是分析130例Ph-MPNs患者中PH的患病率、临床特征及相关危险因素，探讨PH对患者预后的影响，为临床识别不良预后因素及早期预防和治疗PH提供参考。纳入了2020年1月1日至2024年12月31日在北京安贞医院接受治疗的130例Ph-MPNs患者。通过超声心动图（ECHO）评估PH风险，以三尖瓣反流速度（TRV）>2.8 m/s作为PH高风险的标准。收集患者的一般信息、血液学指标、生化指标、基因突变及超声心动图数据并进行统计分析。130例Ph-MPNs患者中PH的总体患病率为15.4%。原发性骨髓纤维化（PMF）患者的PH患病率最高（72.3%），显著高于真性红细胞增多症（PV）患者（9.9%）和原发性血小板增多症（ET）患者（10.4%）（P<0.05）。PH高风险组患者年龄较大，血红蛋白水平较低，脾肿大和继发性骨髓纤维化的患病率较高。PH高风险组患者的死亡率显著高于正常风险组（20%对1.81%，P=0.0004）。多因素Cox回归分析显示，高龄（HR=1.029，P=0.0332）和PH高风险（HR=1.034，P=0.0432）是患者生存的独立危险因素。Logistic回归分析显示，血红蛋白降低（OR=0.9657，P=0.0062）、脾肿大（OR=5.105，P=0.0413）和继发性骨髓纤维化（OR=7.959，P=0.0321）是PH高风险的独立危险因素。本研究揭示了Ph-MPNs患者中PH的患病率、危险因素及其预后意义。提示在Ph-MPNs患者治疗过程中定期监测相关危险因素的变化，警惕并预防PH，对改善患者预后具有重要临床意义。
