Combined chelation with high-dose deferiprone and deferoxamine to improve survival and restore cardiac function effectiv
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ORIGINAL ARTICLE
Combined chelation with high-dose deferiprone and deferoxamine to improve survival and restore cardiac function effectively in patients with transfusion-dependent thalassemia presenting severe cardiac complications Tzu-Yao Chuang 1 & Ju-Pi Li 2,3 & Te-Fu Weng 2 & Kang-Hsi Wu 2,3 & Yu-Hua Chao 2,3 Received: 11 April 2020 / Accepted: 20 July 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Iron overload–induced cardiomyopathy is the leading cause of death in patients with transfusion-dependent thalassemia (TDT). The mortality is extremely high in these patients with severe cardiac complications, and how to rescue them remains a challenge. It is reasonable to use combined chelation with deferiprone (L1) and deferoxamine (DFO) because of their shuttle and synergistic effects on iron chelation. Here, seven consecutive patients with TDT who had severe cardiac complications between 2002 and 2019 and received combined chelation therapy with oral high-dose L1 (100 mg/kg/day) and continuous 24-h DFO infusion (50 mg/kg/day) in our hospital were reported. Survival for eight consecutive patients receiving DFO monotherapy for their severe cardiac complications between 1984 and 2001 was compared. We found that combined chelation therapy with high-dose L1 and DFO was efficient to improve survival and cardiac function in patients with TDT presenting severe cardiac complications. Reversal of arrhythmia to sinus rhythm was noted in all patients. Their 1-month follow-up left ventricular ejection fraction increased significantly (P < 0.001). There were no deaths, and all patients were discharged from hospital with good quality of life. In contrast, all the eight patients receiving DFO monotherapy died (P < 0.001). Accordingly, combined chelation therapy with high-dose L1 and DFO should be considered in patients with TDT presenting cardiac complications. Keywords Deferiprone . Deferoxamine . Cardiac complications . Iron overload . Thalassemia
Introduction Iron overload–induced cardiomyopathy remains the major cause of death in patients needing long-term red blood cell (RBC) transfusion, such as those with transfusion-dependent thalassemia (TDT). The mortality is extremely high in these patients presenting severe cardiac complications like decompensated heart failure and arrhythmia. How to rescue them from the critical condition is urgently important, but it remains a real challenge [1]. There are three iron chelators currently available
in the world, including deferoxamine (DFO), deferiprone (L1), and deferasirox [2]. The use of either one may be insufficient under such circumstances. L1 was found to have better cardioprotection, and shuttle and synergistic effects on iron chelation were demonstrated when concurrently used with DFO [2–6]. Therefore, it is reasonable to enhance iron clearance and restore cardiac function by using combined intensive chelation with L1 and DFO. Since 2002, combined chelation therapy with high-dose L1 and DFO has been used to treat patients with TDT who had seve
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