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Thalassaemia—A global view

Authors :
Peter Hokland
Shahina Daar
Wael Khair
Sujit Sheth
Ali T. Taher
Lorenza Torti
Chattree Hantaweepant
Deborah Rund
Source :
Hokland, P, Daar, S, Khair, W, Sheth, S, Taher, A T, Torti, L, Hantaweepant, C & Rund, D 2023, ' Thalassaemia-A global view ', British Journal of Haematology, vol. 201, no. 2, pp. 199-214 . https://doi.org/10.1111/bjh.18671
Publication Year :
2023
Publisher :
Wiley, 2023.

Abstract

The thalassaemias are a group of genetic disorders of haemoglobin which are endemic in the tropics but are now found worldwide due to migration. Basic standard of care therapy includes regular transfusions to maintain a haemoglobin level of around 10 g/dL, together with iron chelation therapy to prevent iron overload. Novel therapies, bone marrow transplantation, and gene therapy are treatment options that are unavailable in many countries with stressed economies. This Wider Perspectives article presents the strategies for management of an adolescent refugee patient with beta thalassaemia, as it would be performed by expert haematologists in six countries: Italy, Lebanon, Oman, the Sudan, Thailand and the United States. The experienced clinicians in each country have adapted their practice according to the resources available, which vary greatly. Even in the current modern era, providing adequate transfusions and chelation is problematic in many countries. On the other hand, ensuring adherence to therapy, particularly during adolescence, is a similar challenge seen in all countries. The concluding section highlights the disparities in available therapies and puts the role of novel therapies into a societal context.

Details

ISSN :
13652141 and 00071048
Volume :
201
Database :
OpenAIRE
Journal :
British Journal of Haematology
Accession number :
edsair.doi.dedup.....a73d65c2dc0203fb029a21b5674f381c
Full Text :
https://doi.org/10.1111/bjh.18671