在输血依赖型地中海贫血患者中,磁共振成像监测不同器官铁水平的重要性是什么?

IF 2.3 4区 医学 Q2 HEMATOLOGY
Expert Review of Hematology Pub Date : 2025-04-01 Epub Date: 2025-03-31 DOI:10.1080/17474086.2025.2486379
Antonella Meloni, Vincenzo Positano, Paolo Ricchi, Alessia Pepe, Riccardo Cau
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引用次数: 0

摘要

铁超载是输血依赖性地中海贫血(TDT)中器官损伤的主要病理生理驱动因素。磁共振成像(MRI)提供了详细的见解分布和严重程度的铁积累在不同的器官。涵盖领域:本专题报告描述了MRI对TDT患者的临床和治疗管理以及短期和长期结果的影响。检索PubMed、Scopus和b谷歌Scholar数据库,确定2024年11月之前发表的相关研究。专家意见:心脏和肝脏磁共振成像现在是公认的模式,融入临床实践。它们已成为定制铁螯合疗法,以特定患者的需要和监测治疗效果必不可少的。心脏铁负荷控制的改善转化为发病率和死亡率的降低。在资源有限的情况下,MRI的可及性仍然有限,该领域的进展依赖于教育和培训中心,以确保准确的执行和解释。建立了胰铁水平评估的临床病理意义、预后价值和可重复性,为其临床应用开辟了道路。关于肾脏、肾上腺和垂体铁沉积的资料有限,需要更多的研究来充分确立其功能意义,并规范和验证MRI方案。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
What is the importance of monitoring iron levels in different organs over time with magnetic resonance imaging in transfusion-dependent thalassemia patients?

Introduction: Iron overload is the main pathophysiological driver of organ damage in transfusion-dependent thalassemia (TDT). Magnetic resonance imaging (MRI) provides detailed insights into the distribution and severity of iron accumulation in the different organs.

Areas covered: This special report describes the impact of MRI on clinical and therapeutic management and short- and long-term outcomes in TDT patients. PubMed, Scopus, and Google Scholar databases were searched to identify the relevant studies published before November 2024.

Expert opinion: Cardiac and hepatic MRI are now well-established modalities, integrated into the clinical practice. They have become essential for tailoring iron chelation therapies to the specific patient's needs and for monitoring treatment efficacy. The improved control of cardiac iron burden has translated into reduced morbidity and mortality. The MRI accessibility remains limited in resource-limited settings and progress in this field relies on educating and training centers to ensure accurate execution and interpretation. The clinicopathological significance, prognostic value, and reproducibility of pancreatic iron levels assessment have been established, charting a path toward its clinical use. There are limited data about renal, adrenal, and pituitary iron deposition, and more research is needed to fully establish the functional significance and to standardize and validate the MRI protocols.

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来源期刊
CiteScore
4.70
自引率
3.60%
发文量
98
审稿时长
6-12 weeks
期刊介绍: Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.
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