Cabantchik Z Ioav, Breuer William, Zanninelli G, Cianciulli P
Department of Biological Chemistry, Institute of Life Sciences, Hebrew University of Jerusalem, Jerusalem, 91904 Israel.
Best Pract Res Clin Haematol. 2005 Jun;18(2):277-87. doi: 10.1016/j.beha.2004.10.003.
Labile plasma iron (LPI) represents a component of non-transferrin-bound iron (NTBI) that is both redox-active and chelatable, capable of permeating into organs and inducing tissue iron overload. It appears in various types of hemosiderosis (transfusional and non-transfusional) and in other iron-overload conditions. Sustained levels of LPI could over time compromise organ (e.g. heart) function and patient survival. With the advent of methods for measuring LPI in the clinical setting, it has become possible to assess the implications of LPI in the management of iron overload based on regimens of iron chelation. As LPI is detected primarily in patients with transfusional iron overload and other forms of hemosiderosis, we review here regimens of iron chelation with deferrioxamine and deferiprone (separately or combined) in terms of their efficacy in minimizing daily exposure to LPI in thalassemia major and thalassemia intermedia patients.
不稳定血浆铁（LPI）是非转铁蛋白结合铁（NTBI）的一个组成部分，具有氧化还原活性且可螯合，能够渗透到器官中并导致组织铁过载。它出现在各种类型的血色素沉着症（输血性和非输血性）以及其他铁过载病症中。持续的LPI水平随着时间推移可能会损害器官（如心脏）功能和患者生存。随着临床环境中测量LPI方法的出现，基于铁螯合方案评估LPI在铁过载管理中的影响成为可能。由于LPI主要在输血性铁过载患者和其他形式的血色素沉着症患者中被检测到，我们在此回顾去铁胺和去铁酮（单独或联合使用）的铁螯合方案，就其在最大限度减少重型地中海贫血和中间型地中海贫血患者每日LPI暴露方面的疗效进行阐述。
