Prognosis and duration

Thalassaemia major

Without regular transfusions children with beta-Thalassaemia major die before the age of five from heart failure or infection. Successful survival into adulthood depends on close monitoring of the affected person in a specialist Thalassaemia centre where all the physical, psychological, genetic and social effects of the condition can be addressed.

From a therapeutic stand point successful treatment includes avoidance of iron overload through chelation therapy. Without treatment of iron overload death from heart disease occurs in the early twenties. Life expectancy in adults is reduced due to liver and/or heart disease secondary to iron overload.

Bone marrow transplantation is more likely to be successful if undertaken before the age of sixteen, and when the marrow is donated by a close relative, usually a sibling, who has a good genetic match to the recipient. The prognosis is less favourable for the recipient with beta-Thalassaemia complications such as liver damage and iron overload.

Cord blood transfusion is a relatively new procedure and there is little data at present on long-term outcomes.

Thalassaemia intermedia

Children and adults with Thalassaemia intermedia have a better prognosis. Individuals with more severe levels of anaemia are likely to have reduced life expectancy and poor overall health due to complications of the condition.

Thalassaemia minor/carrier

People are asymptomatic, lead normal lives and have a normal life expectancy.

Amended May 2009