Abstract
BackgroundThe 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).MethodsThe 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.ResultsThe 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).ConclusionSince 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 care.
Highlights
Introduction major has increased throughout NorthWest Europe, Over the past 50 years the prevalence of thalassaemia reflecting immigration from endemic areas [1]
The reliable identification of cardiac siderosis was made possible with the T2* cardiovascular magnetic resonance (CMR) technique which became available in the United Kingdom (UK) in 1999 [12], and this has been a driver for changes in, and intensification of, iron chelator therapy
The control of cardiac iron overload as the major cause of death in thalassaemia major has been greatly improved by the deployment of T2* CMR, which makes cardiac siderosis visible both to clinicians and patients, in combination with appropriate alterations in iron chelator treatment
Summary
Introduction major has increased throughout NorthWest Europe, Over the past 50 years the prevalence of thalassaemia reflecting immigration from endemic areas [1]. United Kingdom (UK) a database of affected children was created in the 1960s to assess the prevalence of the disorder among Cypriots in London [2], and to describe its natural history [3] This was subsequently developed into the UK Thalassaemia Register, a clinical and research tool designed to promote equitable high quality care by simultaneously collecting audit data, and disseminating specialist knowledge to clinics with small numbers of patients. The oral iron chelating agent deferiprone was first used in clinical practice in 1987 [10], and its use in the UK has gradually increased, for patients who cannot tolerate deferoxamine treatment [11], and for patients with cardiac iron loading. A study of survival and causes of death was undertaken which aimed to include the possible impact of T2* cardiovascular magnetic resonance (CMR)
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