肺高血压(pulmonary hypertension,PH)是指肺内循环系统发生高血压,伴或不伴有肺小动脉病变的恶性肺血管疾病,往往引起右心功能衰竭甚至死亡。血液系统疾病如溶血性贫血、慢性骨髓增生性疾病、浆细胞病常合并有PH。针对不同的临床情况,其致病因素及治疗方案又有所不同。文章将对诊治过程中可能合并PH的几种血液系统疾病分别从病理生理学基础、临床特点、治疗及预后等方面进行阐述。 Pulmonary hypertension (PH) is a type of malignant pulmonary vascular disease that causes hypertension in the pulmonary circulation, with or without pulmonary arterioles, often causing right heart failure or even death. Hematological diseases such as hemolytic anemia, chronic myeloproliferative diseases, plasma cell disease often associated with PH. For different clinical situations, the risk factors and treatment options are different. The article will elaborate the pathophysiology, clinical features, treatment and prognosis of several hematologic diseases that may be combined with PH during the diagnosis and treatment.