Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort
Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort
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DOI：
10.1182/blood-2011-04-344507
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发表时间：
2012-03-22
期刊：
影响因子：
20.3
通讯作者：
Neufeld, Ellis J.
中科院分区：
文献类型：
--
作者：
Kwiatkowski, Janet L.;Kim, Hae-Young;Neufeld, Ellis J.
地中海贫血的发病率和死亡率与铁负荷有关。器官特异性铁成像的最新进展和口服地拉罗司的可用性有望改善临床护理，但这些资源的使用程度和目前的螯合实践尚未得到很好的描述。在本研究中，我们研究了2002-2011年327例输血依赖性地中海贫血受试者（平均入组年龄为22.1 ± 2.5岁）的螯合使用和铁测量值的变化，平均随访时间为8.0年（范围为4.4-9.0年）。目前使用的主要螯合剂是地拉罗司，其次是去铁胺，然后是联合治疗。肝脏和心脏磁共振成像的使用增加了5倍以上（P <15 mg/g干重，和/或心脏T2*
Morbidity and mortality in thalassemia are associated with iron burden. Recent advances in organ-specific iron imaging and the availability of oral deferasirox are expected to improve clinical care, but the extent of use of these resources and current chelation practices have not been well described. In the present study, we studied chelation use and the change in iron measurements in 327 subjects with transfusion-dependent thalassemia (mean entry age, 22.1 +/- 2.5 years) from 2002-2011, with a mean follow-up of 8.0 years (range, 4.4-9.0 years). The predominant chelator currently used is deferasirox, followed by deferoxamine and then combination therapies. The use of both hepatic and cardiac magnetic resonance imaging increased more than 5-fold (P15 mg/g dry weight, and/or cardiac T2*