Division of Hematology and Oncology, Department of Internal Medicine, University of Cincinnati, 231 Albert Sabin Way, Cincinnati, OH 45267-0562, USA.
Hematol Oncol Clin North Am. 2010 Dec;24(6):1131-44. doi: 10.1016/j.hoc.2010.08.001. Epub 2010 Sep 29.
Reactivation of fetal hemoglobin (HbF) expression is an important therapeutic option in adult patients with hemoglobin disorders. The understanding of the developmental regulation of γ-globin gene expression was followed by the identification of a number of chemical compounds that can reactivate HbF synthesis in vitro and in vivo in patients with hemoglobin disorders. These HbF inducers can be grouped in several classes based on their mechanisms of action. This article focuses on pharmacologic agents that were tested in humans and discusses current knowledge about the mechanisms by which they induce HbF.
重新激活胎儿血红蛋白 (HbF) 的表达是治疗成人血红蛋白疾病的重要方法。在了解γ-珠蛋白基因表达的发育调控后,人们鉴定出了许多可在体外和体内重新激活血红蛋白疾病患者 HbF 合成的化合物。这些 HbF 诱导剂可根据其作用机制分为几类。本文重点介绍了已在人类中进行测试的药物,并讨论了它们诱导 HbF 的机制的现有知识。