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

doi:10.1007/s00277-020-04196-y

中山醫學大學機構典藏 CSMUIR > 中山醫學大學研究成果 > 期刊論文 > Item 310902500/24411

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题名:	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
作者:	Chuang, TY;Li, JP;Weng, TF;Wu, KH;Chao, YH
关键词:	Deferiprone;Deferoxamine;Cardiac complications;Iron overload;Thalassemia
日期:	2020
上传时间:	2022-08-09T08:01:51Z (UTC)
出版者:	SPRINGER
ISSN:	0939-5555
摘要:	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.
URI:	http://dx.doi.org/10.1007/s00277-020-04196-y https://www.webofscience.com/wos/woscc/full-record/WOS:000554339000002 https://ir.csmu.edu.tw:8080/handle/310902500/24411
關聯:	ANNALS OF HEMATOLOGY ,2020 ,v99 ,issue 10 ,p2289-2294
显示于类别:	[中山醫學大學研究成果] 期刊論文

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