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Studies in haemoglobin E beta-thalassaemia

Show simple item record Olivieri, N. F. en_US Muraca, G. M. en_US O Donnell, A. en_US Premawardhena, A. en_US Fisher, C. en_US Weatherall, D. J. en_US 2014-10-29T09:27:00Z 2014-10-29T09:27:00Z 2008 en_US
dc.identifier.citation British Journal of Haematology. 2008; 141(3): pp.388-97 en_US
dc.identifier.issn 0007-1048 (Print) en_US
dc.identifier.issn 1365-2141 (Electronic) en_US
dc.description.abstract Haemoglobin E beta-thalassaemia is the commonest form of severe thalassaemia in many Asian countries, but little is known about its natural history, the reasons for its clinical diversity, or its optimal management. Despite its frequency, haemoglobin E beta-thalassaemia is often managed in an ill-defined and haphazard way, usually by demand transfusion. We studied a cohort of Sri Lankan patients with haemoglobin Ebeta-thalassaemia over 5 years, and identified several genetic and environmental factors possibly contributing to the phenotypic diversity of the disorder. These included modifiers of haemoglobin F production, malaria and age-related changes in adaptation to anaemia. Our findings suggest that in many patients, haemoglobin E beta-thalassaemia can be managed without transfusion, even with low haemoglobin levels. Age-related changes in the pattern of adaptation to anaemia suggest that more cost-effective approaches to management should be explored. en_US
dc.publisher Wiley-Blackwell en_US
dc.subject Thalassemia en_US
dc.subject beta-Thalassemia en_US
dc.subject beta-Thalassemia-genetics en_US
dc.subject Hemoglobin E en_US
dc.subject Hemoglobin E-genetics en_US
dc.subject Sri Lanka en_US
dc.title Studies in haemoglobin E beta-thalassaemia en_US
dc.type Review Article en_US
dc.identifier.department Medicine en_US

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