Pietrangelo Antonello
Center for Hemochromatosis, University Hospital of Modena and Reggio Emilia, Modena, Italy.
Am J Hematol. 2007 Dec;82(12 Suppl):1142-6. doi: 10.1002/ajh.21101.
The effects of systemic iron overload in hereditary (e.g., classic HFE hemochromatosis) or acquired disorders (e.g., transfusion-dependent iron overload) are well known. Several other iron overload diseases, with an observed mild-to-moderate increase in iron in selected organs (e.g., the liver or the brain), or with "misdistribution" of iron within cells (e.g., reticuloendothelial cells) or subcellular organelles (e.g., mitochondria), have been recognized more recently. The deleterious impact of any excess iron may be high as active redox iron may directly contribute to cell damage or affect signaling pathways involved in cell necrosis-apoptosis or organ fibrosis and cancer. This article discusses the potential use of iron chelation therapy to treat iron overload from causes other than transfusion overload.
遗传性疾病(如经典的HFE血色素沉着症)或后天性疾病(如输血依赖型铁过载铁过载)中全身铁过载的影响是众所周知的。最近还认识到其他几种铁过载疾病,这些疾病在某些器官(如肝脏或大脑)中观察到铁有轻度至中度增加,或者铁在细胞(如网状内皮细胞)或亚细胞器(如线粒体)内“分布异常”。任何过量铁的有害影响可能很大,因为活性氧化还原铁可能直接导致细胞损伤,或影响参与细胞坏死-凋亡或器官纤维化和癌症的信号通路。本文讨论了铁螯合疗法在治疗输血过载以外原因引起的铁过载方面的潜在用途。