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Studies in haemoglobin E beta-thalassaemia.
Br J Haematol 2008; 141(3):388-97BJ

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 E beta-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.

Authors+Show Affiliations

Hemoglobinopathy Research Program, Toronto General Hospital, University of Toronto, Toronto, ON, Canada. noliv@attglobal.netNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18410572

Citation

Olivieri, Nancy F., et al. "Studies in Haemoglobin E Beta-thalassaemia." British Journal of Haematology, vol. 141, no. 3, 2008, pp. 388-97.
Olivieri NF, Muraca GM, O'Donnell A, et al. Studies in haemoglobin E beta-thalassaemia. Br J Haematol. 2008;141(3):388-97.
Olivieri, N. F., Muraca, G. M., O'Donnell, A., Premawardhena, A., Fisher, C., & Weatherall, D. J. (2008). Studies in haemoglobin E beta-thalassaemia. British Journal of Haematology, 141(3), pp. 388-97. doi:10.1111/j.1365-2141.2008.07126.x.
Olivieri NF, et al. Studies in Haemoglobin E Beta-thalassaemia. Br J Haematol. 2008;141(3):388-97. PubMed PMID: 18410572.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Studies in haemoglobin E beta-thalassaemia. AU - Olivieri,Nancy F, AU - Muraca,Giulia M, AU - O'Donnell,Angela, AU - Premawardhena,Anuja, AU - Fisher,Christopher, AU - Weatherall,David J, PY - 2008/4/16/pubmed PY - 2008/6/28/medline PY - 2008/4/16/entrez SP - 388 EP - 97 JF - British journal of haematology JO - Br. J. Haematol. VL - 141 IS - 3 N2 - 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 E beta-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. SN - 1365-2141 UR - https://www.unboundmedicine.com/medline/citation/18410572/Studies_in_haemoglobin_E_beta_thalassaemia_ L2 - https://doi.org/10.1111/j.1365-2141.2008.07126.x DB - PRIME DP - Unbound Medicine ER -