铁超载:β-地中海贫血症的致命弱点。

IF 1.4 4区 医学 Q4 HEMATOLOGY Transfusion Clinique et Biologique Pub Date : 2024-06-06 DOI:10.1016/j.tracli.2024.06.001
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引用次数: 0

摘要

在发达国家,系统输血加上铁螯合疗法大大延长了地中海贫血患者的寿命。由于人类机体没有清除过量铁的保护机制,因此铁超载是地中海贫血症的一个重要问题,会导致器官损伤,尤其是心脏和肝脏。因此,铁螯合疗法对于预防或逆转器官铁超载至关重要。目前有三种广泛使用的铁螯合剂,可作为单一疗法或联合疗法。铁螯合剂的选择取决于多种因素,包括当地指南、药物供应情况和个人临床情况。尽管治疗取得了进步,但挑战依然存在,尤其是在资源有限的环境中,这凸显了改善全球医疗服务的必要性。本综述讨论了地中海贫血的临床管理、当前治疗方法和未来发展方向,重点关注铁过载及其并发症。此外,它还强调了在将地中海贫血转变为一种可控的慢性疾病方面所取得的进展,以及新型疗法进一步改善患者预后的潜力。
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Iron overload: The achilles heel of β-thalassemia

Systematic transfusions coupled with iron chelation therapy have substantially improved the life expectancy of thalassemia patients in developed nations. As the human organism does not have a protective mechanism to remove excess iron, iron overload is a significant concern in thalassemia, leading to organ damage, especially in the heart and liver. Thus, iron chelation therapy is crucial to prevent or reverse organ iron overload. There are three widely used iron chelators, either as monotherapy or in combination. The choice of iron chelator depends on several factors, including local guidelines, drug availability, and the individual clinical scenario. Despite treatment advancements, challenges persist, especially in resource-limited settings, highlighting the need for improved global healthcare access. This review discusses clinical management, current treatments, and future directions for thalassemia, focusing on iron overload and its complications. Furthermore, it underscores the progress in transforming thalassemia into a manageable chronic condition and the potential of novel therapies to further enhance patient outcomes.

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来源期刊
CiteScore
2.50
自引率
11.80%
发文量
234
审稿时长
36 days
期刊介绍: Transfusion Clinique et Biologique, the official journal of the French Society of Blood Transfusion (SFTS): - an aid to training, at a European level - the only French journal indexed in the hematology and immunology sections of Current Contents Transfusion Clinique et Biologique spans fundamental research and everyday practice, with articles coming from both sides. Articles, reviews, case reports, letters to the editor and editorials are published in 4 editions a year, in French or in English, covering all scientific and medical aspects of transfusion: immunology, hematology, infectious diseases, genetics, molecular biology, etc. And finally, a convivial cross-disciplinary section on training and information offers practical updates. Readership: "Transfusers" are many and various: anesthetists, biologists, hematologists, and blood-bank, ICU and mobile emergency specialists...
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