Digital Repository, University of Kelaniya Medicine Journal/Magazine Articles

 
Please use this identifier to cite or link to this item: http://repository.kln.ac.lk/handle/123456789/1985
Title: Adaptation to anemia in hemoglobin E-beta thalassemia
Authors: Allen, A.
Fisher, C.
Premawardhena, A.
Peto, T.
Allen, S.J.
Arambepola, M.
Thayalsuthan, V.
Olivieri, N.
Weatherall, D.
Keywords: beta-Thalassemia
Anemia-blood
Hemoglobin E-metabolism
Oxygen-metabolism
beta-Thalassemia-blood
beta-Thalassemia-complications
Fetal Hemoglobin-analysis
Issue Date: 2010
Publisher: American Society of Hematology
Citation: Blood. 2010; 116(24): pp.5368-70
Abstract: Hemoglobin E beta thalassemia is the commonest form of severe thalassemia in many Asian countries. Its remarkably variable clinical phenotype presents a major challenge to determining its most appropriate management. In particular, it is not clear why some patients with this condition can develop and function well at very low hemoglobin levels. Here, we demonstrate that patients with hemoglobin E beta thalassemia have a significant decrease in the oxygen affinity of their hemoglobin, that is an increased P(50) value, in response to anemia. This may in part reflect the lower level of hemoglobin F in this condition compared with other forms of beta thalassemia intermedia. The ability to right-shift the oxygen dissociation curve was retained across the spectrum of mild and severe phenotypes, despite the significantly higher levels of hemoglobin F in the former, suggesting that efforts directed at producing a modest increase in the level of hemoglobin F in symptomatic patients with this disease should be of therapeutic value.
Description: Indexed in MEDLINE
URI: http://repository.kln.ac.lk/handle/123456789/1985
ISSN: 0006-4971 (Print)
1528-0020 (Electronic)
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