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

Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ - J Cardiovasc Magn Reson (2008)

Bottom Line: 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).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.

View Article: PubMed Central - HTML - PubMed

Affiliation: UCL Centre for Health Informatics and Multiprofessional Education, Holborn Union Building, Whittington Campus, Highgate Hill, London, N19 5LW, UK. b.modell@pcps.ucl.ac.uk

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, alongside other improvements in clinical care.

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Comparison of life expectancy for patients who were alive at the beginning of 1970, 1980, 1990 and 2000, based on mortality in the subsequent five years (four years for the period 2000–2003). Curves are constructed by calculating the proportion of patients in each group who were alive at the time indicated and were still alive at the end of the next 5 years. For the four year interval 2000–2003, mortality was multiplied by 1.25 to adjust to 5 years. The calculation shows an average life-expectancy of 17 years in 1970, 27 years in 1980 and 37 years in 1990. Since 2000 over 80% of patients have a life expectancy of more than 40 years. It is still not possible to estimate ultimate life-expectancy, and the prognosis for older patients remains "open-ended".
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Figure 2: Comparison of life expectancy for patients who were alive at the beginning of 1970, 1980, 1990 and 2000, based on mortality in the subsequent five years (four years for the period 2000–2003). Curves are constructed by calculating the proportion of patients in each group who were alive at the time indicated and were still alive at the end of the next 5 years. For the four year interval 2000–2003, mortality was multiplied by 1.25 to adjust to 5 years. The calculation shows an average life-expectancy of 17 years in 1970, 27 years in 1980 and 37 years in 1990. Since 2000 over 80% of patients have a life expectancy of more than 40 years. It is still not possible to estimate ultimate life-expectancy, and the prognosis for older patients remains "open-ended".

Mentions: Survival data is often presented as Kaplan Meier curves, which show cumulative mortality in patient cohorts born in specified periods [7,20,33], but this focus on attrition rate can be pessimistic, especially in clinical discussions with older patients. In practice, the more optimistic picture of the current life-expectancy of living patients, as calculated by life insurance companies, is more useful for patients and health workers. Figure 2 compares life expectancy for patients who were alive at the beginning of 1970, 1980, 1990 and 2000, based on mortality in the subsequent five years (four years for the period 2000–2003). The calculation shows an average life-expectancy of 17 years in 1970, 27 years in 1980 and 37 years in 1990. Since 2000, over 80% of patients have a life expectancy of more than 40 years. It is still not possible to estimate ultimate life-expectancy, and the prognosis for older patients remains "open-ended".


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

Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ - J Cardiovasc Magn Reson (2008)

Comparison of life expectancy for patients who were alive at the beginning of 1970, 1980, 1990 and 2000, based on mortality in the subsequent five years (four years for the period 2000–2003). Curves are constructed by calculating the proportion of patients in each group who were alive at the time indicated and were still alive at the end of the next 5 years. For the four year interval 2000–2003, mortality was multiplied by 1.25 to adjust to 5 years. The calculation shows an average life-expectancy of 17 years in 1970, 27 years in 1980 and 37 years in 1990. Since 2000 over 80% of patients have a life expectancy of more than 40 years. It is still not possible to estimate ultimate life-expectancy, and the prognosis for older patients remains "open-ended".
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2563008&req=5

Figure 2: Comparison of life expectancy for patients who were alive at the beginning of 1970, 1980, 1990 and 2000, based on mortality in the subsequent five years (four years for the period 2000–2003). Curves are constructed by calculating the proportion of patients in each group who were alive at the time indicated and were still alive at the end of the next 5 years. For the four year interval 2000–2003, mortality was multiplied by 1.25 to adjust to 5 years. The calculation shows an average life-expectancy of 17 years in 1970, 27 years in 1980 and 37 years in 1990. Since 2000 over 80% of patients have a life expectancy of more than 40 years. It is still not possible to estimate ultimate life-expectancy, and the prognosis for older patients remains "open-ended".
Mentions: Survival data is often presented as Kaplan Meier curves, which show cumulative mortality in patient cohorts born in specified periods [7,20,33], but this focus on attrition rate can be pessimistic, especially in clinical discussions with older patients. In practice, the more optimistic picture of the current life-expectancy of living patients, as calculated by life insurance companies, is more useful for patients and health workers. Figure 2 compares life expectancy for patients who were alive at the beginning of 1970, 1980, 1990 and 2000, based on mortality in the subsequent five years (four years for the period 2000–2003). The calculation shows an average life-expectancy of 17 years in 1970, 27 years in 1980 and 37 years in 1990. Since 2000, over 80% of patients have a life expectancy of more than 40 years. It is still not possible to estimate ultimate life-expectancy, and the prognosis for older patients remains "open-ended".

Bottom Line: 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).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.

View Article: PubMed Central - HTML - PubMed

Affiliation: UCL Centre for Health Informatics and Multiprofessional Education, Holborn Union Building, Whittington Campus, Highgate Hill, London, N19 5LW, UK. b.modell@pcps.ucl.ac.uk

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, alongside other improvements in clinical care.

Show MeSH
Related in: MedlinePlus