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对于依赖输血的地中海贫血患者,随着时间推移通过磁共振成像监测不同器官中铁含量的重要性是什么?

What is the importance of monitoring iron levels in different organs over time with magnetic resonance imaging in transfusion-dependent thalassemia patients?

作者信息

Meloni Antonella, Positano Vincenzo, Ricchi Paolo, Pepe Alessia, Cau Riccardo

机构信息

Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.

Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Napoli, Italy.

出版信息

Expert Rev Hematol. 2025 Apr;18(4):291-299. doi: 10.1080/17474086.2025.2486379. Epub 2025 Mar 31.

DOI:10.1080/17474086.2025.2486379
PMID:40152085
Abstract

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.

摘要

引言

铁过载是依赖输血的地中海贫血(TDT)器官损伤的主要病理生理驱动因素。磁共振成像(MRI)能详细洞察不同器官中铁蓄积的分布和严重程度。

涵盖领域

本专题报告描述了MRI对TDT患者临床和治疗管理以及短期和长期结局的影响。检索了PubMed、Scopus和谷歌学术数据库,以识别2024年11月之前发表的相关研究。

专家观点

心脏和肝脏MRI现已成为成熟的检查方式,并融入临床实践。它们对于根据特定患者的需求调整铁螯合疗法以及监测治疗效果至关重要。对心脏铁负荷的更好控制已转化为发病率和死亡率的降低。在资源有限的环境中,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读。胰腺铁水平评估的临床病理意义、预后价值和可重复性已经确立,为其临床应用指明了方向。关于肾脏、肾上腺和垂体铁沉积的数据有限,需要更多研究来充分确立其功能意义,并标准化和验证MRI方案。

相似文献

1
What is the importance of monitoring iron levels in different organs over time with magnetic resonance imaging in transfusion-dependent thalassemia patients?对于依赖输血的地中海贫血患者,随着时间推移通过磁共振成像监测不同器官中铁含量的重要性是什么?
Expert Rev Hematol. 2025 Apr;18(4):291-299. doi: 10.1080/17474086.2025.2486379. Epub 2025 Mar 31.
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Quantitative MRI evaluation of iron deposition in patients with transfusion-dependent thalassemia: clinical management insights.输血依赖型地中海贫血患者铁沉积的定量MRI评估:临床管理见解
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Iron Chelation Therapy as a Modality of Management.铁螯合疗法作为一种治疗方式。
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