Pulmonary hypertension associated with myeloproliferative disorders: A retrospective study of ten cases
Pulmonary hypertension associated with myeloproliferative disorders: A retrospective study of ten cases
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DOI：
10.1159/000112822
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发表时间：
2008-01-01
期刊：
影响因子：
3.7
通讯作者：
Hermine, O.
中科院分区：
文献类型：
--
作者：
Guilpain, P.;Montani, D.;Hermine, O.
背景资料：肺动脉高压（PH）是一种严重的血液动力学疾病，其中肺动脉压力持续升高，导致右侧心力衰竭。一些研究表明PH和骨髓增生性疾病（MPD）之间存在关联。目的：本研究描述PH合并MPD的临床、血液学和血流动力学特征。研究方法：我们回顾性分析了10例PH合并MPD的病例：真性红细胞增多症（8例）和原发性血小板增多症（2例），随访时间为1993年至2002年。通过右侧心脏导管插入术、通气/灌注肺扫描和肺血管造影（如需要）确定基线评价。结果如下：6例患者确诊为慢性血栓栓塞性肺动脉高压（CTEPH），4例患者患有与MPD相关的肺动脉高压（PAH），无其他PAH风险因素。CTEPH和PAH合并MPD的血流动力学特征相似。所有病例中CTEPH的诊断与MPD的诊断同时发生（5例真性红细胞增多症和1例原发性血小板增多症）。与MPD相关的PAH发生在MPD进展的后期（3例真性红细胞增多症和1例原发性血小板增多症），中位时间为MPD诊断后162个月，并且与髓样化生相关（p <0.01）。结论：我们在MPD的背景下描述了2种不同形式的PH：CTEPH，在MPD的早期诊断，PAH，在MPD的后期发生，与髓样化生相关。MPD患者的呼吸困难逐渐加重需要进一步研究以排除PAH和CTEPH，而CTEPH的诊断需要排除MPD。版权所有（C）2007 S. Karger AG，巴塞尔。
Background: Pulmonary hypertension (PH) is a severe hemodynamic disorder in which the pulmonary artery pressure is persistently elevated, leading to right-sided heart failure. Some studies have suggested an association between PH and myeloproliferative diseases (MPD). Objectives: This study describes clinical, hematological and hemodynamic characteristics of PH associated with MPD. Methods: We retrospectively reviewed 10 cases of PH associated with MPD: polycythemia vera ( 8 patients) and essential thrombocythemia ( 2 patients), followed between 1993 and 2002. The baseline evaluation was established by right-sided heart catheterization, ventilation/perfusion lung scan and pulmonary angiography if required. Results: Six patients had confirmed chronic thromboembolic pulmonary hypertension (CTEPH) and 4 had pulmonary arterial hypertension (PAH) associated with MPD without other risk factors for PAH. The hemodynamic characteristics of CTEPH and PAH associated with MPD were similar. The diagnosis of CTEPH was concomitant to that of MPD in all cases ( 5 polycythemia vera and 1 essential thrombocythemia). The PAH associated with MPD occurred later in the evolution of the MPD ( 3 polycythemia vera and 1 essential thrombocythemia) with a median of 162 months after the diagnosis of MPD, and it was associated with myeloid metaplasia ( p < 0.01). Conclusion: We describe 2 distinct forms of PH in the context of MPD: CTEPH, which is diagnosed at an early stage of the MPD, and PAH, which occurs later in the course of the MPD and is associated with myeloid metaplasia. Progressively increasing dyspnea in a patient with an MPD warrants further investigation to rule out PAH and CTEPH, while a diagnosis of CTEPH warrants ruling out MPD. Copyright (C) 2007 S. Karger AG, Basel.