Pulmonary Institute, Rabin Medical Center, Petach Tiqwa 49100, Israel.
Respir Med. 2012 Apr;106(4):594-8. doi: 10.1016/j.rmed.2011.12.005. Epub 2012 Jan 13.
Pulmonary arterial hypertension (PAH) has been associated with hemolytic conditions such as sickle cell disease but the possible role of hemolysis in the pathogenesis or pathophysiology of other forms of PAH has not been studied. Erythrocyte lifespan is the gold-standard test of hemolysis and may be measured by assaying erythrocyte creatine (EC) levels. EC decreases as the erythrocyte ages, so patients with hemolysis have high EC levels.
We measured EC and other parameters of hemolysis in patients with idiopathic and connective tissue associated PAH and normal controls.
In patients with PAH (n = 40), EC levels were higher than in controls n = 30 (patients EC 1.72 mcmol/g HgB 95%CI[1.51, 1.96], controls EC 1.05 mcmol/g HgB [0.93, 1.19], p < 0.0001). High levels of EC correlated with worse 6 min walk (r = -0.42, p < 0.0001) and worse functional class (p = 0.002). Other indirect indices of hemolysis (total lactate dehydrogenase, red cell distribution width) were also increased in patients with PAH relative to controls.
There is evidence of subclinical hemolysis in patients with PAH, and higher levels of hemolysis are associated with poorer exercise capacity.
肺动脉高压（PAH）与镰状细胞病等溶血性疾病有关，但溶血性在其他形式 PAH 的发病机制或病理生理学中的可能作用尚未得到研究。红细胞寿命是溶血性的金标准检测，可以通过检测红细胞肌酸（EC）水平来测量。随着红细胞的老化，EC 会降低，因此溶血性患者的 EC 水平较高。
我们测量了特发性和结缔组织相关 PAH 患者以及正常对照者的 EC 和其他溶血性参数。
在 PAH 患者（n = 40）中，EC 水平高于对照组（n = 30）（患者 EC 1.72 mcmol/g HgB [1.51, 1.96]，对照组 EC 1.05 mcmol/g HgB [0.93, 1.19]，p < 0.0001）。高水平的 EC 与较差的 6 分钟步行距离（r = -0.42，p < 0.0001）和较差的功能分级（p = 0.002）相关。PAH 患者的其他间接溶血性指标（总乳酸脱氢酶、红细胞分布宽度）也高于对照组。
PAH 患者存在亚临床溶血性的证据，较高水平的溶血性与运动能力较差相关。
