Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance
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BioMed Central
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Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance Bernadette Modell*1, Maren Khan1, Matthew Darlison1, Mark A Westwood2, David Ingram1 and Dudley J Pennell2 Address: 1UCL Centre for Health Informatics and Multiprofessional Education (CHIME), Holborn Union Building, Whittington Campus, Highgate Hill, London, N19 5LW, UK and 2CMR Unit, National Heart and Lung Institute, Imperial College & Royal Brompton Hospital, London, SW3 6NP, UK Email: Bernadette Modell* - [email protected]; Maren Khan - [email protected]; Matthew Darlison - [email protected]; Mark A Westwood - [email protected]; David Ingram - [email protected]; Dudley J Pennell - [email protected] * Corresponding author
Published: 25 September 2008 Journal of Cardiovascular Magnetic Resonance 2008, 10:42
doi:10.1186/1532-429X-10-42
Received: 11 September 2008 Accepted: 25 September 2008
This article is available from: http://www.jcmr-online.com/content/10/1/42 © 2008 Modell et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Background: The UK Thalassaemia Register records births, deaths and selected clinical data of patients with thalassaemia who are resident in the UK. A study of survival and causes of death was undertaken which aimed to include the possible impact of T2* cardiovascular magnetic resonance (CMR). Methods: The Register was updated to the end of 2003, copies of death certificates were obtained, and causes of death in beta thalassaemia major were extracted. In addition, patients who had T2* CMR assessment of cardiac iron load and/or received the oral iron chelator deferiprone were identified from clinical records. Results: The main causes of death were anaemia (before 1980), infections, complications of bone marrow transplantation and cardiac disease due to iron overload. From 1980 to 1999 there were 12.7 deaths from all causes per 1,000 patient years. Forty per cent of patients born before 1980 had T2* cardiovascular magnetic resonance between 2000 and 2003, and 36% of these patients were prescribed deferiprone before end of 2003. In 2000–2003, the death rate from all causes fell significantly to 4.3 per 1,000 patient years (-62%, p < 0.05). This was mainly driven by the reduction in the rate of deaths from iron overload which fell from 7.9 to 2.3 deaths per 1,000 patient years (-71%, p < 0.05). Conclusion: Since 1999, there has been a marked improvement in survival in thalassaemia major in the UK, which has been mainly driven by a reduction in deaths due to cardiac iron overload. The most likely causes for this include the introduction of T2* CMR to identify myocardial siderosis and appropriate intensification of iron chelation treatment, alongsi
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