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Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance

Authors: Bernadette Modell*1, Maren Khan1, Matthew Darlison1, Mark A Westwood2,David Ingram1 and Dudley J Pennell2

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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
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, alongside other improvements in clinical