Document Detail

Adaptation to anemia in hemoglobin E-ß thalassemia.
MedLine Citation:
PMID:  20833979     Owner:  NLM     Status:  MEDLINE    
Hemoglobin E β 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 Eβ 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 β 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.
Angela Allen; Christopher Fisher; Anuja Premawardhena; Timothy Peto; Stephen Allen; Mahinda Arambepola; Vivekanandan Thayalsutha; Nancy Olivieri; David Weatherall
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-09-10
Journal Detail:
Title:  Blood     Volume:  116     ISSN:  1528-0020     ISO Abbreviation:  Blood     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-14     Completed Date:  2011-01-06     Revised Date:  2012-02-01    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  5368-70     Citation Subset:  AIM; IM    
Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK.
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MeSH Terms
Adaptation, Physiological
Anemia / blood*
Biological Transport
Fetal Hemoglobin / analysis
Hemoglobin E / analysis,  metabolism*
Oxygen / metabolism*
Protein Binding
beta-Thalassemia / blood*,  complications*
Grant Support
//Wellcome Trust
Reg. No./Substance:
7782-44-7/Oxygen; 9034-61-1/Hemoglobin E; 9034-63-3/Fetal Hemoglobin
Erratum In:
Blood. 2011 Dec 22;118(26):6994-5

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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