Peng Ching-Tien, Tsai Chang-Hai, Wu Kang-Hsi
Department of Laboratory Medicine, China Medical University & Hospital, Taichung, Taiwan.
Hemoglobin. 2008;32(1-2):49-62. doi: 10.1080/03630260701680078.
The iron chelators deferoxamine (DFO) and deferiprone (L1) have demonstrated their ability to normalize cardiac function in patients with iron overload-induced cardiac disease. However, conventional chelation with subcutaneous DFO fails to prevent iron deposition in two-thirds of thalassemia major patients, placing them at risk of heart failure and its complications. Deferiprone appears to be more effective in cardiac iron removal. The detection and management of heart complications have improved dramatically over the last 7 years. Non invasive techniques of quantifying iron burden via magnetic resonance imaging (MRI) have been validated. A better understanding of cardiac pathophysiology and improved ability to detect at-risk populations are yielding better outcomes and reduced morbidity. We continue to appraise readily available bedside tools for monitoring thalassemia patients with heart complications, and here we summarize studies from the literature and our own findings. Deferiprone chelation was found to be of statistically significant benefit in upgrading cardiac function and reducing iron accumulation. The use of echocardiography and MRI to closely monitor cardiac functions associated with iron overload complications and mortality has proved quite practical.
铁螯合剂去铁胺（DFO）和去铁酮（L1）已证明其能够使铁过载所致心脏病患者的心脏功能恢复正常。然而，三分之二的重型地中海贫血患者采用皮下注射DFO的传统螯合疗法无法防止铁沉积，这使他们面临心力衰竭及其并发症的风险。去铁酮在清除心脏铁方面似乎更有效。在过去7年中，心脏并发症的检测和管理有了显著改善。通过磁共振成像（MRI）定量铁负荷的非侵入性技术已得到验证。对心脏病理生理学的更好理解以及检测高危人群能力的提高，正带来更好的治疗效果并降低发病率。我们继续评估用于监测有心脏并发症的地中海贫血患者的现有床边工具，在此我们总结文献研究和我们自己的发现。发现去铁酮螯合在改善心脏功能和减少铁蓄积方面具有统计学显著益处。事实证明，使用超声心动图和MRI密切监测与铁过载并发症及死亡率相关的心脏功能非常实用。
