慢性骨髓增殖性疾病并发肺动脉高压4例临床分析

Clinical analysis of 4 cases with chronic myeloproliferative disorders complicated by pulmonary hypertension

目的加强对慢性骨髓增殖性疾病（CMPD）患者合并肺动脉高压的认识，以期临床早期诊断和治疗，改善预后。方法回顾性分析4例慢性骨髓增殖性疾病并发肺动脉高压患者的临床及实验室检查资料、治疗及转归。结果4例患者诊断慢性骨髓增殖性疾病时年龄为44～72岁，诊断原发病到发现肺动脉高压1～26年。4例患者临床均表现为乏力、活动耐力下降，脾脏明显肿大，均有不同程度的贫血，3例患者有血小板减少。诊断肺动脉高压时肺动脉压力为58～83mmHg，三尖瓣反流速率3．5～4．3m／s。4例患者诊断CMPD后均接受正规治疗，随诊过程中发现肺动脉高压，3例患者于诊断肺动脉高压1～2年内死亡。结论肺动脉高压是慢性骨髓增殖性疾病患者常见的心血管并发症，目前治疗方法有限，慢性骨髓增殖性疾病患者病程中出现肺动脉高压提示预后不良。

Objective To increase awareness of chronic myeloproliferative disorders （CMPD） complicated by pulmonary hypertension, make the patients to be diagnosed and treated earlier and improve the prognosis. Methods Retrospective analysis of the clinical course, laboratory result, treatment and out- come of 4 cases, who had chronic myeloproliferative disorders with pulmonary hypertension. Results The age of 4 patients was from 44 to 72 years old when they were diagnosed of chronic myeloproliferative disor- ders. The interval between the diagnosis of chronic myeloproliferative disorders to confirmation of pulmona- ry hypertension ranged from 1 to 26 years. All patients had fatigue, activity intolerance, and splenomegaly. Laboratory examination revealed all patients had anemia in different degree and thrombopenia in three pa- tients. The pulmonary pressure was from 58 mmHg to 80mmHg, and the tricuspid regurgitation velocity was from 3.5 m/s to 4.3 m/s. All patients had received regular treatment after the diagnosis of CMPD. During the follow-up,pulmonary hypertension were found. Three patients died 1 to 2 years after the diagnosis of pulmonary hypertension. Conclusion Pulmonary hypertension is the common cardiovascular complication of patients with chronic myeloproliferative disorders. Currently efficacious treatments are limited. Its emergence imply the poor prognosis.