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一名依赖输血的地中海贫血患者在短暂不依从铁螯合治疗后,心脏和肝脏中铁快速蓄积。

Rapid iron loading in heart and liver in a patient with transfusion dependent thalassaemia after brief poor compliance with iron chelation therapy.

作者信息

Daar Shahina, Ahmed Saeed, Berdoukas Vasili

机构信息

Department of Haematology, College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman.

出版信息

Sultan Qaboos Univ Med J. 2010 Dec;10(3):401-4. Epub 2010 Nov 14.

PMID:21509263
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3074730/
Abstract

Iron loading in patients with transfusion dependent thalassaemia is considered to occur primarily in the liver and, once the liver becomes saturated, other organs begin loading. We report here a splenectomised male patient who was treated for hepatitis C virus infection. Prior to starting antiviral therapy, his serum ferritin was maintained below 500 ng/ml with deferiprone monotherapy; cardiac T2* by magnetic resonance imaging was 48.8ms and hepatic T2* was 19.5ms. After twelve months of antiviral treatment during which time he was very poorly compliant with his deferoxamine chelation therapy, his ferritin had risen to 3820 ng/ml and cardiac and hepatic T2* findings were 12.7 ms and 14.5 ms respectively, indicating increased iron loading in both organs, but particularly in the heart. Fifteen months after recommencing combination chelation, his ferritin was 95 ng/ml and cardiac and hepatic T2* were 27.5 and 28.4ms respectively, indicating complete clearance of iron load in both organs. This case demonstrates that iron overload can develop rapidly and in some cases there is relatively rapid iron loading in the heart as compared to the liver.

摘要

依赖输血的地中海贫血患者的铁负荷被认为主要发生在肝脏,一旦肝脏饱和,其他器官便开始蓄积铁。我们在此报告一名接受过脾切除术的男性患者,他曾接受丙型肝炎病毒感染的治疗。在开始抗病毒治疗之前,他通过去铁酮单药治疗将血清铁蛋白维持在500 ng/ml以下;磁共振成像显示心脏T2为48.8ms,肝脏T2为19.5ms。在抗病毒治疗的十二个月期间,他对去铁胺螯合疗法的依从性很差,其铁蛋白升至3820 ng/ml,心脏和肝脏的T2结果分别为12.7 ms和14.5 ms,表明两个器官的铁负荷均增加,但心脏尤为明显。重新开始联合螯合治疗十五个月后,他的铁蛋白为95 ng/ml,心脏和肝脏的T2分别为27.5和28.4ms,表明两个器官的铁负荷已完全清除。该病例表明铁过载可能迅速发展,在某些情况下,与肝脏相比,心脏的铁蓄积相对较快。

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本文引用的文献

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Br J Haematol. 2010 Feb;148(3):466-75. doi: 10.1111/j.1365-2141.2009.07970.x. Epub 2009 Nov 12.
2
Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major.心脏T2*磁共振成像预测重型地中海贫血患者心脏并发症的研究
Circulation. 2009 Nov 17;120(20):1961-8. doi: 10.1161/CIRCULATIONAHA.109.874487. Epub 2009 Oct 2.
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T2* cardiovascular magnetic resonance in the management of thalassemia patients in Oman.阿曼地中海贫血患者管理中的T2*心血管磁共振成像
Haematologica. 2009 Jan;94(1):140-1. doi: 10.3324/haematol.13845. Epub 2008 Nov 10.
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Longitudinal analysis of heart and liver iron in thalassemia major.重型地中海贫血中心脏和肝脏铁含量的纵向分析。
Blood. 2008 Oct 1;112(7):2973-8. doi: 10.1182/blood-2008-04-148767. Epub 2008 Jul 23.
5
Rapid iron loading in a pregnant woman with transfusion-dependent thalassemia after brief cessation of iron chelation therapy.一名依赖输血的地中海贫血孕妇在短暂停止铁螯合治疗后出现快速铁负荷增加。
Eur J Haematol. 2008 Aug;81(2):157-9. doi: 10.1111/j.1600-0609.2008.01092.x. Epub 2008 May 6.
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Haematologica. 2007 Jan;92(1):131-2. doi: 10.3324/haematol.10455.
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