Wahl Shannon, Vichinsky Elliott
Department of Hematology/Oncology, Children's Hospital and Research Center Oakland 747 52nd Street, Oakland, CA 94609 USA.
F1000 Med Rep. 2010 Feb 11;2:10. doi: 10.3410/M2-10.
Pulmonary hypertension (PH) has been reported with nearly all forms of the inherited as well as the acquired hemolytic anemias. Recent research investigating the pathophysiology of PH in sickle cell disease and thalassemia has helped elucidate the central role of hemolysis-mediated endothelial dysfunction in the development of PH in these populations. Although the most appropriate treatment of PH in patients with hemolytic anemia is not clearly defined, the associated significant increased risk of death underscores the need for randomized clinical trials in this area.
几乎所有形式的遗传性和获得性溶血性贫血都曾有肺动脉高压（PH）的报道。近期针对镰状细胞病和地中海贫血中PH病理生理学的研究，有助于阐明溶血介导的内皮功能障碍在这些人群PH发生过程中的核心作用。尽管溶血性贫血患者PH的最恰当治疗方法尚未明确界定，但相关的死亡风险显著增加凸显了该领域进行随机临床试验的必要性。
