Haemoglobin E beta thalassaemia in Sri Lanka


Journal article


Anuja Premawardhena, Christopher Fisher, Nancy F Olivieri, Shanthimala de Silva, Mahinda Arambepola, Wilson Perera, Angela O’Donnell, Tim Peto, Vip Viprakasit, Laura Merson, Giulia M Muraca, David J Weatherall
Lancet, vol. 366(9495), 2005, pp. 1467-70


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APA   Click to copy
Premawardhena, A., Fisher, C., Olivieri, N. F., de Silva, S., Arambepola, M., Perera, W., … Weatherall, D. J. (2005). Haemoglobin E beta thalassaemia in Sri Lanka. Lancet, 366(9495), 1467–1470. https://doi.org/10.1016/S0140-6736(05)67396-5


Chicago/Turabian   Click to copy
Premawardhena, Anuja, Christopher Fisher, Nancy F Olivieri, Shanthimala de Silva, Mahinda Arambepola, Wilson Perera, Angela O’Donnell, et al. “Haemoglobin E Beta Thalassaemia in Sri Lanka.” Lancet 366, no. 9495 (2005): 1467–70.


MLA   Click to copy
Premawardhena, Anuja, et al. “Haemoglobin E Beta Thalassaemia in Sri Lanka.” Lancet, vol. 366, no. 9495, 2005, pp. 1467–70, doi:10.1016/S0140-6736(05)67396-5.


BibTeX   Click to copy

@article{anuja2005a,
  title = {Haemoglobin E beta thalassaemia in Sri Lanka},
  year = {2005},
  issue = {9495},
  journal = {Lancet},
  pages = {1467-70},
  volume = {366},
  doi = {10.1016/S0140-6736(05)67396-5},
  author = {Premawardhena, Anuja and Fisher, Christopher and Olivieri, Nancy F and de Silva, Shanthimala and Arambepola, Mahinda and Perera, Wilson and O’Donnell, Angela and Peto, Tim and Viprakasit, Vip and Merson, Laura and Muraca, Giulia M and Weatherall, David J}
}

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 clinical diversity, or its management. We studied 109 Sri Lankan patients with the disorder over 5 years. 25 patients were not receiving transfusion; transfusion was stopped with no deleterious effect in a further 37. We identified several genetic and environmental factors that might contribute to the phenotypic diversity of the disorder, including modifiers of haemoglobin F production, malaria, and age-related changes in adaptive function. Our findings suggest that haemoglobin E beta thalassaemia can be managed without transfusion in many patients, even with low haemoglobin levels. Age-related changes in the pattern of adaptation to anaemia suggest that different and more cost-effective approaches to management should be explored.