Combined therapy with deferiprone and desferrioxamine successfully regresses severe heart failure in patients with β-thalassemia major
Combined therapy with deferiprone and desferrioxamine successfully regresses severe heart failure in patients with β-thalassemia major
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DOI：
10.1007/s00277-003-0820-0
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发表时间：
2004-07
影响因子：
3.5
通讯作者：
Kang-Hsi Wu;Jeng‐Sheng Chang;C. Tsai;C. Peng
中科院分区：
文献类型：
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作者：
Kang-Hsi Wu;Jeng‐Sheng Chang;C. Tsai;C. Peng
铁沉积引起的心脏并发症是重型β地中海贫血患者死亡的主要原因。去铁酮（L1）被发现在消耗心肌铁方面比去铁胺（DFX）具有更大的功效。此外，L1和DFX的联合治疗产生了累加或协同的铁螯合作用。我们报告了两例重型β地中海贫血合并严重心力衰竭患者的成功治疗。磁共振图像显示心脏信号强度明显恢复，表明心脏铁负荷显著减少。未观察到显著不良反应。因此，对于重型β地中海贫血合并心脏并发症的患者，应考虑L1和DFX联合治疗。
Cardiac complications caused by iron deposition are major causes of death in patients with β-thalassemia major. Deferiprone (L1) was found to have greater efficacy at depleting myocardial iron than desferrioxamine (DFX). Furthermore, combined therapy with L1 and DFX produced an additive or synergistic iron chelating effect. We report the successful treatment of severe heart failure in two patients with β-thalassemia major with the combined therapy. Magnetic resonance images showed a marked recovery of signal intensity in the heart, indicating a significant reduction of iron load in the heart. No significant adverse effects were noted. Therefore, combined therapy with L1 and DFX should be considered in patients with β-thalassemia major and cardiac complications.