Document Detail


Adaptation to anemia in hemoglobin E-ß thalassemia.
MedLine Citation:
PMID:  20833979     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Angela Allen; Christopher Fisher; Anuja Premawardhena; Timothy Peto; Stephen Allen; Mahinda Arambepola; Vivekanandan Thayalsutha; Nancy Olivieri; David Weatherall
Related Documents :
3469969 - Management of splenectomy failures in chronic immune thrombocytopenic purpura: role of ...
7446549 - The liver in sickle cell disease. a clinicopathologic study of 70 patients.
8122949 - Acute chest syndrome in adult afro-caribbean patients with sickle cell disease. analysi...
136229 - Safety of long-term tetracycline therapy for acne.
15797499 - Salmonella typhi osteitis can mimic tuberculosis. a report of three cases.
20938329 - Does erythrocyte blood transfusion prevent acute kidney injury?: propensity-matched cas...
24119609 - Prognosis for sixth nerve palsy arising from paranasal sinus disease.
12825119 - Aerosolized pentamidine prophylaxis against aids-related pneumocystis carinii pneumonia...
504969 - Circulating megakaryocytes in patients with pulmonary inflammation and in patients subj...
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    
Affiliation:
Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adaptation, Physiological
Anemia / blood*
Biological Transport
Fetal Hemoglobin / analysis
Hemoglobin E / analysis,  metabolism*
Humans
Oxygen / metabolism*
Protein Binding
beta-Thalassemia / blood*,  complications*
Grant Support
ID/Acronym/Agency:
//Wellcome Trust
Chemical
Reg. No./Substance:
7782-44-7/Oxygen; 9034-61-1/Hemoglobin E; 9034-63-3/Fetal Hemoglobin
Comments/Corrections
Erratum In:
Blood. 2011 Dec 22;118(26):6994-5

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


Previous Document:  Progenitor cell dose determines the pace and completeness of engraftment in a xenograft model for co...
Next Document:  In pediatric patients, age has more impact on dosing of vitamin K antagonists than VKORC1 or CYP2C9 ...