费城染色体阴性的骨髓增殖性肿瘤患者肺动脉高压发生率、危险因素及预后分析

Analysis of Incidence Rate,Risk Factors and Prognosis of Pulmo-nary Hypertension in Ph^(-) MPNs Patients

目的:探讨并分析费城染色体阴性的骨髓增殖性肿瘤患者肺动脉高压(PH)的发病率、影响因素和对预后的影响。方法:通过回顾性分析271例费城染色体阴性骨髓增殖性肿瘤患者的临床资料,区分不同疾病亚型分类,依据是否发生PH分为PH^(+)和PH^(-)两组,应用统计学方法,分析患者PH的发病率、危险因素和对预后的影响。结果:271例患者中PH的总体发病率为26.9%且根据疾病亚型分类后发现,原发性骨髓纤维化患者的PH发病率明显高于真性红细胞增多症患者和原发性血小板增多症患者(均P<0.05);多因素回归分析结果显示,高龄、长病程、JAK2阳性和升高的红细胞压积、乳酸脱氢酶、单核细胞计数、尿酸水平是费城染色体阴性骨髓增殖性肿瘤患者发生PH的独立危险因素(OR>1,P<0.05),且不同疾病亚型的独立危险因素存在一定的差异;生存分析结果显示,除原发性血小板增多症患者外其他类型患者中PH^(+)患者的总生存率明显低于PH~-患者(均P<0.05)。结论:PH在费城染色体阴性骨髓增殖性肿瘤患者中发病率较高,其危险因素是多样的且合并PH的患者总体生存率降低,因此,临床上应高度警惕此类患者PH的发生。

Objective: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).Methods: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.Results: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 P<0.05).Multivariate regression analysis showed that advanced age,long disease course,JAK2 positive and increased hematocrit,lactate dehydrogenase,monocyte count,and uric acid level were independent risk factors for PH in Ph-MPNs patients(OR>1,P<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 P<0.05).Concdusion: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.