Thalassaemia—A global view
Issued Date
2023-04-01
Resource Type
ISSN
00071048
eISSN
13652141
Scopus ID
2-s2.0-85148438158
Pubmed ID
36799486
Journal Title
British Journal of Haematology
Volume
201
Issue
2
Start Page
199
End Page
214
Rights Holder(s)
SCOPUS
Bibliographic Citation
British Journal of Haematology Vol.201 No.2 (2023) , 199-214
Suggested Citation
Hokland P., Daar S., Khair W., Sheth S., Taher A.T., Torti L., Hantaweepant C., Rund D. Thalassaemia—A global view. British Journal of Haematology Vol.201 No.2 (2023) , 199-214. 214. doi:10.1111/bjh.18671 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/82093
Title
Thalassaemia—A global view
Other Contributor(s)
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.