Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Am J Hematol. 2012 Feb;87(2):221-3. doi: 10.1002/ajh.22228. Epub 2011 Nov 25.
Chronic transfusion reduces the risk of recurrent stroke in children with sickle cell anemia (SCA) but leads to iron loading. Management of transfusional iron overload in SCA has been reported as suboptimal [1], but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial. Data obtained during screening, including past and entry liver iron concentration (LIC) measurements, ferritin values, and chelation were analyzed. The mean age at enrollment was 12.9 ± 4 years and the mean duration of transfusion was 7 ± 3.8 years. Baseline LIC (median 12.94 mg/g dw) and serum ferritin (median 3,164 ng/mL) were elevated. Chelation therapy was initiated after a mean of 2.6 years of transfusions. At study entry, 137 were receiving chelation, most of whom (90%) were receiving deferasirox. This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA.
慢性输血可降低镰状细胞贫血（SCA）患儿复发性中风的风险，但会导致铁过载。SCA 患者输血性铁过载的管理被报道为不理想[1]，但缺乏描述 SCA 患儿铁过载监测和治疗实践的研究，特别是近年来螯合剂选择的扩大。我们调查了 161 名曾因中风接受输血的 SCA 患儿的铁负荷程度和治疗实践，这些患儿参与了Stroke with Transfusions Changing to Hydroxyurea（SWiTCH）试验。分析了筛选期间获得的数据，包括既往和入组时的肝脏铁浓度（LIC）测量值、铁蛋白值和螯合作用。入组时的平均年龄为 12.9 ± 4 岁，输血的平均持续时间为 7 ± 3.8 年。基线 LIC（中位数 12.94mg/g dw）和血清铁蛋白（中位数 3164ng/mL）升高。输血后平均 2.6 年开始螯合治疗。在研究入组时，有 137 人正在接受螯合治疗，其中大多数（90%）人正在接受地拉罗司。这项研究强调了需要更好地监测慢性输血 SCA 患儿的铁负荷，并及时调整治疗。
