Morris Claudia R, Kuypers Frans A, Kato Gregory J, Lavrisha Lisa, Larkin Sandra, Singer Titi, Vichinsky Elliott P
Department of Emergency Medicine, Children's Hospital & Research Center at Oakland, 747 52nd Street, Oakland, California 94609, USA.
Ann N Y Acad Sci. 2005;1054:481-5. doi: 10.1196/annals.1345.058.
Accumulating evidence supports the existence of a condition involving hemolysis-associated pulmonary hypertension (PHT). Hemolysis-induced release of cell-free hemoglobin and red blood cell arginase, resulting in impaired nitric oxide bioavailability, endothelial dysfunction, and PHT, has been reported in sickle cell disease. Since thalassemia is also a condition of chronic hemolysis, these patients are at risk. The data demonstrate that hemolysis-induced dysregulation of arginine metabolism and PHT also occurs in thalassemia. Erythrocyte release of arginase during hemolysis contributes to the development of PHT. Therapies that maximize arginine and nitric oxide bioavailability may benefit patients with thalassemia.
越来越多的证据支持存在一种与溶血相关的肺动脉高压（PHT）病症。在镰状细胞病中，已经报道了溶血诱导的游离血红蛋白和红细胞精氨酸酶的释放，导致一氧化氮生物利用度受损、内皮功能障碍和PHT。由于地中海贫血也是一种慢性溶血病症，这些患者有患病风险。数据表明，溶血诱导的精氨酸代谢失调和PHT也发生在地中海贫血中。溶血过程中红细胞释放精氨酸酶有助于PHT的发展。使精氨酸和一氧化氮生物利用度最大化的疗法可能使地中海贫血患者受益。
