慢性骨髓增殖性疾病并发肺动脉高压的临床特点和预后分析

Clinical characterization and prognosis of chronic myeloproliferative disorders combined with pulmonary artery high pressure

目的:探讨慢性骨髓增殖性疾病(CMPD)并发肺动脉高压(PAH)的临床特点及PAH对于CMPD临床预后的影响,进一步丰富CMPD的评估体系。方法:选取CMPD并发PAH病例11例,选取同期CMPD肺动脉压正常病例27例作为对照。结果:与肺动脉压正常的CMPD病例相比,CMPD并发PAH平均年龄及血小板计数明显升高,且有更高的血栓发生概率,预后更差。另外2组间亚病种构成比显著不同,CMPD并发PAH中原发性血小板增多症所占比例较高。结论:高龄、高血小板计数、伴发有血栓形成、诊断ET等均是CMPD可能并发PAH的危险因素。对于诊断明确的病例,积极控制PAH是改善这类患者预后的关键。

Objective：The aims of this study were to investigate the clinical characteristics of chronic myeloproliferative disorders（CMPD） combined with pulmonary artery high pressure（PAH） and the influence of PAH on the prognosis of CMPD,and to enrich the assessment system of CMPD.Method：Eleven cases of CMPD combined with PAH diagnosed were retrospectively analyzed.Twenty-seven cases of CMPD without PAH during the same period were taken as control.Result：Compared to the cases of CMPD with normal pulmonary artery pressure,average age and platelet counts of the cases of CMPD combined with PAH significantly increased,（P0.05）.There were higher incidence of thrombosis（P0.05）and worse prognosis in the cases of CMPD combined with PAH.Proportions of sub-disease between the two groups were significantly different（P0.05）.Conclusion：The old age,high platelet count,thrombosis,diagnosis of ET are risk factors associated with PAH for patients diagnosed as CMPD.Actively controlling the pulmonary hypertension is the key to improve the prognosis of those patients diagnosed as CMPD combined with PAH.