Thalassaemia-A global view.

Autor: Hokland P; Faculty of Health, Department of Clinical Medicine, Aarhus University, Aarhus, Denmark., Daar S; College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman., Khair W; Khartoum Oncology Hospital, Khartoum, Sudan., Sheth S; Division of Hematology Oncology, Department of Pediatrics, Weill Cornell Medicine, New York City, New York, USA., Taher AT; Division of Hematology & Oncology, Department of Internal Medicine, American University of Beirut Medical Centre, Beirut, Lebanon., Torti L; Hemoglobinopathies Unit, Hematology Department, S. Eugenio Hospital, (ASL Roma 2), Rome, Italy., Hantaweepant C; Faculty of Medicine Siriraj Hospital, Division of Hematology, Department of Medicine, Mahidol University, Bangkok, Thailand., Rund D; Department of Haematology, Hebrew University-Hadassah Medical Centre, Jerusalem, Israel.
Jazyk: angličtina
Zdroj: British journal of haematology [Br J Haematol] 2023 Apr; Vol. 201 (2), pp. 199-214. Date of Electronic Publication: 2023 Feb 17.
DOI: 10.1111/bjh.18671
Abstrakt: 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.
(© 2023 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)
Databáze: MEDLINE
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