Table.
Present status of 796 UK patients with βthalassaemia major
Cohort Patients Deaths (%) Age at death
25 y
1945–54 25 21 (84) 8 12 1
1955–64 119 67 (56) 19 32 16
1965–74 188 63 (34) 14 37 12
1975–84 237 33 (14) 13 20 0
1985–94 179 5 (3) 5 0 0
1995 onwards 48 1 (2) 1 0 0
Total 796 190 (24) 60 101 29
Table options
The figure shows Kaplan-Meier survival curves for 736 patients who survived beyond 12 years of age, by 10-year birth cohort. A progressive change in the curve is expected, reflecting the evolution of treatment and extent of patient adherence.
Survival beyond 12 years of age by 10-year birth cohort
Figure.
Survival beyond 12 years of age by 10-year birth cohort
Figure options
1945–54 birth cohort—Members of this cohort should now be more than 45 years of age. 75% of survivors to age 12 years died of iron overload, mostly before …show more content…
Some were started on desferrioxamine in early childhood, others only in their teens or not at all. About 30% died before 24 years of age and 20% died between 24 and 35 years of age, indicating inadequate chelation. A plateau at 50% survival to 40 years of age suggests successful control of iron overload in about half the patients, and confirms that long-term survival is possible even with organ damage due to prior iron overload, hepatitis C infection, or both.
1965–74 birth cohort—Members of this cohort should now be between 25 and 35 years of age. Most started iron-chelation therapy when still young so this cohort should show the full benefit of treatment. However, over 30% have died and the survival curve resembles that for the previous cohort, displaced 8 years to the right. There is no sign of a plateau. It seems likely that the curve will converge with that of the previous cohort at about 50% survival.
1975–84 birth cohort—These patients should now be between 15 and 25 years. To date, their survival resembles that of the previous two
Present status of 796 UK patients with βthalassaemia major
Cohort Patients Deaths (%) Age at death
25 y
1945–54 25 21 (84) 8 12 1
1955–64 119 67 (56) 19 32 16
1965–74 188 63 (34) 14 37 12
1975–84 237 33 (14) 13 20 0
1985–94 179 5 (3) 5 0 0
1995 onwards 48 1 (2) 1 0 0
Total 796 190 (24) 60 101 29
Table options
The figure shows Kaplan-Meier survival curves for 736 patients who survived beyond 12 years of age, by 10-year birth cohort. A progressive change in the curve is expected, reflecting the evolution of treatment and extent of patient adherence.
Survival beyond 12 years of age by 10-year birth cohort
Figure.
Survival beyond 12 years of age by 10-year birth cohort
Figure options
1945–54 birth cohort—Members of this cohort should now be more than 45 years of age. 75% of survivors to age 12 years died of iron overload, mostly before …show more content…
Some were started on desferrioxamine in early childhood, others only in their teens or not at all. About 30% died before 24 years of age and 20% died between 24 and 35 years of age, indicating inadequate chelation. A plateau at 50% survival to 40 years of age suggests successful control of iron overload in about half the patients, and confirms that long-term survival is possible even with organ damage due to prior iron overload, hepatitis C infection, or both.
1965–74 birth cohort—Members of this cohort should now be between 25 and 35 years of age. Most started iron-chelation therapy when still young so this cohort should show the full benefit of treatment. However, over 30% have died and the survival curve resembles that for the previous cohort, displaced 8 years to the right. There is no sign of a plateau. It seems likely that the curve will converge with that of the previous cohort at about 50% survival.
1975–84 birth cohort—These patients should now be between 15 and 25 years. To date, their survival resembles that of the previous two