An Hong-Xia, Zhang Qi-Ke, Wei Xiao-Fang, Feng You-Fan, Sun Yan-Qing
First School of Clinical Medical, Gansu University of Chinese Medicine; Lanzhou 730000, Gansu Province, China.
Department of Hematology, Gansu Provincial Hospital; Lanzhou 730000, Gansu Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1463-1471. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.025.
To explore and analyze the incidence rate, influencing factors and impact on prognosis of pulmonary hypertension (PH) in patients with Philadelphia chromosome negative myeloproliferative neoplasms (Ph MPNs).
The clinical data of 271 patients with Ph MPNs were retrospectively analyzed, and different disease subtypes were classified. Patients with different disease types were further divided into PH and PH groups according to whether HP occurred. Statistical methods were used to analyze the incidence rate, risk factors, and impact on prognosis of PH in Ph MPNs patients.
The overall incidence rate of PH among 271 patients was 26.9%, and according to the classification of disease subtypes, it was found that the incidence rate of PH in patients with primary myelofibrosis (PMF) was significantly higher than those of patients with polycythemia vera and essential thrombocythemia (both <0.05). Multivariate regression analysis showed that advanced age, long disease course, positive and increased hematocrit, lactate dehydrogenase, monocyte count, and uric acid level were independent risk factors for PH in Ph MPNs patients ( >1, <0.05), and there were some differences in the independent risk factors between different disease subtypes. Survival analysis results showed that the overall survival (OS) rate of PH patients was significantly lower than that of PH patients in other types except for PMF (all <0.05).
The incidence rate of PH in Ph MPNs patients is high, and its risk factors are diverse. The OS rate of Ph MPNs patients with PH is low. Therefore, we should be highly alert to the occurrence of PH in Ph MPNs patients clinically.
探讨并分析费城染色体阴性骨髓增殖性肿瘤（Ph MPNs）患者肺动脉高压（PH）的发病率、影响因素及其对预后的影响。
回顾性分析271例Ph MPNs患者的临床资料，并对不同疾病亚型进行分类。根据是否发生PH，将不同疾病类型的患者进一步分为PH组和非PH组。采用统计学方法分析Ph MPNs患者中PH的发病率、危险因素及其对预后的影响。
271例患者中PH的总体发病率为26.9%，根据疾病亚型分类发现，原发性骨髓纤维化（PMF）患者中PH的发病率显著高于真性红细胞增多症和原发性血小板增多症患者（均P<0.05）。多因素回归分析显示，高龄、病程长、血细胞比容升高、乳酸脱氢酶升高、单核细胞计数升高及尿酸水平升高是Ph MPNs患者发生PH的独立危险因素（均P>1，P<0.05），且不同疾病亚型的独立危险因素存在一定差异。生存分析结果显示，除PMF外，PH患者的总生存（OS）率显著低于其他类型的非PH患者（均P<0.05）。
Ph MPNs患者中PH的发病率较高，其危险因素多样。Ph MPNs合并PH患者的OS率较低。因此，临床上应高度警惕Ph MPNs患者发生PH。
