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中间型地中海贫血中的铁过载:铁螯合策略的重新评估。

Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies.

机构信息

Department of Internal Medicine, Haematology-Oncology Division, American University of Beirut, Medical Centre, Beirut, Lebanon.

出版信息

Br J Haematol. 2009 Dec;147(5):634-40. doi: 10.1111/j.1365-2141.2009.07848.x. Epub 2009 Aug 13.

Abstract

Thalassaemia intermedia (TI) is a syndrome marked by its diverse underlying genetic basis although its pathophysiology remains unclear, particularly regarding the nature of iron loading and toxicity. It is, however, evident that there are key differences from the extensively studied thalassaemia major (TM) population and caution is required when assessing iron load based on serum ferritin values, as this approach is known to underestimate the true extent of iron loading in patients with TI. Although effective iron chelation therapy has been available for many years, studies in TI-specific populations are rare and evidence suggests that management of iron levels may be less rigorous than in patients with TM and other chronic anaemias. Better understanding of the need to assess and treat iron overload in both transfused and non-transfused TI patients is clearly required.

摘要

中间型地中海贫血(TI)是一种以其不同的潜在遗传基础为特征的综合征,尽管其病理生理学仍不清楚,特别是关于铁负荷和毒性的性质。然而,显然与广泛研究的重型地中海贫血(TM)人群有很大的不同,在根据血清铁蛋白值评估铁负荷时需要谨慎,因为这种方法已知会低估 TI 患者铁负荷的真实程度。尽管有效的铁螯合治疗已经有多年的历史,但针对 TI 特定人群的研究很少,并且有证据表明,铁水平的管理可能不如 TM 和其他慢性贫血患者严格。显然,需要更好地了解评估和治疗输血和非输血 TI 患者铁过载的必要性。

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