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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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Number of deaths of patients with thalassaemia major in the UK by intervals. The number of deaths in the 2000–2003 interval represents deaths during 4 years, and in all the other groups the number of deaths is over 5 years. Iron overload replaced anaemia as the commonest cause of death after 1970, when adequate transfusion schemes became the norm. Iron chelation therapy by subcutaneous infusion of deferoxamine was standard practice after 1980. In 1999, T2* CMR was introduced in the UK, and doctors caring for thalassemia patients were informed of the high cardiac death rate and new options for iron chelation therapy. There has been a 71% reduction in the annualized death-rate from iron overload since 2000.
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Figure 1: Number of deaths of patients with thalassaemia major in the UK by intervals. The number of deaths in the 2000–2003 interval represents deaths during 4 years, and in all the other groups the number of deaths is over 5 years. Iron overload replaced anaemia as the commonest cause of death after 1970, when adequate transfusion schemes became the norm. Iron chelation therapy by subcutaneous infusion of deferoxamine was standard practice after 1980. In 1999, T2* CMR was introduced in the UK, and doctors caring for thalassemia patients were informed of the high cardiac death rate and new options for iron chelation therapy. There has been a 71% reduction in the annualized death-rate from iron overload since 2000.

Mentions: Tables 3, 4, 5 and 6 shows numbers of deaths, number of patient years, and death rates (deaths per 1,000 patient years) from all causes and from iron overload by decade. The final columns of Table 5 and 6 show a steady decrease in death rate from iron overload among patients 10–20 years old. However, in 1990–99 this was offset by an increased death rate among older patients, giving an average death rate from iron overload of 7.9 per 1000 patient years from 1980 to 1999. Since 2000 there has been a marked decrease in deaths from iron overload at any age, giving an average rate of 2.3 per 1,000 patient years (-71%, p < 0.05, figure 1). Table 7 compares average death rates by decade. There was no significant difference in death rates from all causes or from iron overload in the three decades starting in 1970, 1980 and 1990. Since the beginning of 2000 there has been a significant fall in death rate from all causes (-62%, p < 0.05).


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)

Number of deaths of patients with thalassaemia major in the UK by intervals. The number of deaths in the 2000–2003 interval represents deaths during 4 years, and in all the other groups the number of deaths is over 5 years. Iron overload replaced anaemia as the commonest cause of death after 1970, when adequate transfusion schemes became the norm. Iron chelation therapy by subcutaneous infusion of deferoxamine was standard practice after 1980. In 1999, T2* CMR was introduced in the UK, and doctors caring for thalassemia patients were informed of the high cardiac death rate and new options for iron chelation therapy. There has been a 71% reduction in the annualized death-rate from iron overload since 2000.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Number of deaths of patients with thalassaemia major in the UK by intervals. The number of deaths in the 2000–2003 interval represents deaths during 4 years, and in all the other groups the number of deaths is over 5 years. Iron overload replaced anaemia as the commonest cause of death after 1970, when adequate transfusion schemes became the norm. Iron chelation therapy by subcutaneous infusion of deferoxamine was standard practice after 1980. In 1999, T2* CMR was introduced in the UK, and doctors caring for thalassemia patients were informed of the high cardiac death rate and new options for iron chelation therapy. There has been a 71% reduction in the annualized death-rate from iron overload since 2000.
Mentions: Tables 3, 4, 5 and 6 shows numbers of deaths, number of patient years, and death rates (deaths per 1,000 patient years) from all causes and from iron overload by decade. The final columns of Table 5 and 6 show a steady decrease in death rate from iron overload among patients 10–20 years old. However, in 1990–99 this was offset by an increased death rate among older patients, giving an average death rate from iron overload of 7.9 per 1000 patient years from 1980 to 1999. Since 2000 there has been a marked decrease in deaths from iron overload at any age, giving an average rate of 2.3 per 1,000 patient years (-71%, p < 0.05, figure 1). Table 7 compares average death rates by decade. There was no significant difference in death rates from all causes or from iron overload in the three decades starting in 1970, 1980 and 1990. Since the beginning of 2000 there has been a significant fall in death rate from all causes (-62%, p < 0.05).

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