Assistant Professor

Curriculum vitae

Department of Obstetrics and Gynecology

Department of Health Research Methods, Evidence and Impact

McMaster University
1280 Main St. West,
HSC3V - 43B
Hamilton, Ontario ​L8S 4K1

Studies in haemoglobin E beta‐thalassaemia

Journal article

N. Olivieri, G. Muraca, A. O’Donnell, A. Premawardhena, C. Fisher, D. Weatherall
British journal of haematology, 2008

Semantic Scholar DOI PubMed


APA   Click to copy
Olivieri, N., Muraca, G., O’Donnell, A., Premawardhena, A., Fisher, C., & Weatherall, D. (2008). Studies in haemoglobin E beta‐thalassaemia. British Journal of Haematology.

Chicago/Turabian   Click to copy
Olivieri, N., G. Muraca, A. O’Donnell, A. Premawardhena, C. Fisher, and D. Weatherall. “Studies in Haemoglobin E Beta‐Thalassaemia.” British journal of haematology (2008).

MLA   Click to copy
Olivieri, N., et al. “Studies in Haemoglobin E Beta‐Thalassaemia.” British Journal of Haematology, 2008.

BibTeX   Click to copy

  title = {Studies in haemoglobin E beta‐thalassaemia},
  year = {2008},
  journal = {British journal of haematology},
  author = {Olivieri, N. and Muraca, G. and O’Donnell, A. and Premawardhena, A. and Fisher, C. and Weatherall, D.}


Haemoglobin E β‐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 β‐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 β‐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 β‐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.


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