Porter John B, Garbowski Maciej W
Haematology Department, University College London, UCLH and Whittington Hospitals, UCL Cancer Institute, Paul O'Gorman Building, 72 Huntley Street, London WC1E 6BT, UK.
Haematology Department, University College London, Cancer Institute, UCL Cancer Institute, Paul O'Gorman Building, 72 Huntley Street, London WC1E 6BT, UK.
Hematol Oncol Clin North Am. 2018 Apr;32(2):247-259. doi: 10.1016/j.hoc.2017.11.010.
The relationship between blood transfusion intensity, chelatable iron pools, and extrahepatic iron distribution is described in thalassemia. Risk factors for cardiosiderosis are discussed with particular reference to the balance of transfusional iron loading rate and transferrin-iron utilization rate as marked by plasma levels of soluble transferrin receptors. Low transfusion regimens increase residual erythropoiesis allowing for apotransferrin-dependent clearance of non-transferrin-bound iron species otherwise destined for myocardium. The impact of transfusion rates on chelation dosing required for iron balance is also shown.
本文描述了地中海贫血患者输血强度、可螯合铁池与肝外铁分布之间的关系。文中讨论了心脏铁沉着症的危险因素，特别提及了以可溶性转铁蛋白受体血浆水平为标志的输血铁负荷率与转铁蛋白 - 铁利用率之间的平衡。低输血方案可增加残余红细胞生成，从而使脱铁转铁蛋白依赖的非转铁蛋白结合铁物种得以清除，否则这些铁物种会沉积于心肌。文中还展示了输血速率对维持铁平衡所需螯合剂量的影响。
