Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients

Beta-thalassemia/HbE (beta-thal/HbE) is a thalassemia intermedia (TI) which encompasses a broad spectrum of severity. Here, we used deferiprone (DFP) as an iron chelating agent in TI patients receiving intermittent blood transfusion who are asymptomatic for cardiovascular disease in order to evaluat...

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Main Authors: Krittapoom Akrawinthawong, Nithima Chaowalit, Thanasaporn Chatuparisuth, Noppadol Siritanaratkul
Other Authors: Mahidol University
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Published: 2018
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Online Access:https://repository.li.mahidol.ac.th/handle/123456789/12592
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spelling th-mahidol.125922018-05-03T15:34:22Z Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients Krittapoom Akrawinthawong Nithima Chaowalit Thanasaporn Chatuparisuth Noppadol Siritanaratkul Mahidol University Medicine Beta-thalassemia/HbE (beta-thal/HbE) is a thalassemia intermedia (TI) which encompasses a broad spectrum of severity. Here, we used deferiprone (DFP) as an iron chelating agent in TI patients receiving intermittent blood transfusion who are asymptomatic for cardiovascular disease in order to evaluate the effectiveness in iron overload and reduce the possibility of cardiovascular complications. Thirty transfusionin-dependent beta-thal/HbE patients with iron overload were treated with DFP for 1 year. Hematological, biochemical, oxidative stress and echocardiographic parameters were determined. Serum ferritin, nontransferrin-bound iron, and malondialdehyde decreased significantly (P < 0.05) after 1-year treatment with DFP. For echocardiographic results, mean pulmonary arterial pressure and pulmonary vascular resistance were diminished significantly (P < 0.05). All those parameters were still improved after subgroup analysis was done for the high ferritin group ( > 2500 ng/ml). DFP therapy alone improved iron overload and oxidative stress and compliance was good. We propose that prevention of pulmonary hypertension is also possible for TI undergoing intermittent blood transfusion. © W. S. Maney & Son Ltd 2011. 2018-05-03T08:34:22Z 2018-05-03T08:34:22Z 2011-03-01 Article Hematology. Vol.16, No.2 (2011), 113-122 10.1179/102453311X12940641877768 16078454 10245332 2-s2.0-79952658675 https://repository.li.mahidol.ac.th/handle/123456789/12592 Mahidol University SCOPUS https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=79952658675&origin=inward
institution Mahidol University
building Mahidol University Library
continent Asia
country Thailand
Thailand
content_provider Mahidol University Library
collection Mahidol University Institutional Repository
topic Medicine
spellingShingle Medicine
Krittapoom Akrawinthawong
Nithima Chaowalit
Thanasaporn Chatuparisuth
Noppadol Siritanaratkul
Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients
description Beta-thalassemia/HbE (beta-thal/HbE) is a thalassemia intermedia (TI) which encompasses a broad spectrum of severity. Here, we used deferiprone (DFP) as an iron chelating agent in TI patients receiving intermittent blood transfusion who are asymptomatic for cardiovascular disease in order to evaluate the effectiveness in iron overload and reduce the possibility of cardiovascular complications. Thirty transfusionin-dependent beta-thal/HbE patients with iron overload were treated with DFP for 1 year. Hematological, biochemical, oxidative stress and echocardiographic parameters were determined. Serum ferritin, nontransferrin-bound iron, and malondialdehyde decreased significantly (P < 0.05) after 1-year treatment with DFP. For echocardiographic results, mean pulmonary arterial pressure and pulmonary vascular resistance were diminished significantly (P < 0.05). All those parameters were still improved after subgroup analysis was done for the high ferritin group ( > 2500 ng/ml). DFP therapy alone improved iron overload and oxidative stress and compliance was good. We propose that prevention of pulmonary hypertension is also possible for TI undergoing intermittent blood transfusion. © W. S. Maney & Son Ltd 2011.
author2 Mahidol University
author_facet Mahidol University
Krittapoom Akrawinthawong
Nithima Chaowalit
Thanasaporn Chatuparisuth
Noppadol Siritanaratkul
format Article
author Krittapoom Akrawinthawong
Nithima Chaowalit
Thanasaporn Chatuparisuth
Noppadol Siritanaratkul
author_sort Krittapoom Akrawinthawong
title Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients
title_short Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients
title_full Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients
title_fullStr Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients
title_full_unstemmed Effectiveness of deferiprone in transfusionindependent beta-thalassemia/HbE patients
title_sort effectiveness of deferiprone in transfusionindependent beta-thalassemia/hbe patients
publishDate 2018
url https://repository.li.mahidol.ac.th/handle/123456789/12592
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