Haemoglobin E â thalassaemia - the commonest form of severe thalassaemia in many Asian countries - can be managed without transfusion in many patients, suggests a research letter published online today (Thursday September 29, 2005) by The Lancet.
The authors state that the finding has important implications for supplies of donated blood—a scarce resource in many countries.
â-thalassaemia is caused by a mutation in the â-globin gene. Patients with mutations in both of their â-globin genes have thalassaemia major. This form of the disease causes severe transfusion-dependent anaemia. However, in many Asian countries people can inherit one â-thalassaemia gene from one parent and a haemoglobin E gene from the other; a condition called haemoglobin E â thalassaemia. Despite the high prevalence of this disease, little is known about the natural history, reasons for clinical diversity and management of haemoglobin E â thalassaemia. David Weatherall (John Radcliffe Hospital, Oxford, UK) and colleagues studied 109 patients with the disorder at Kurunegala General Hospital, Sri Lanka. 25 patients were not receiving blood transfusions, while the remainder had regular or intermittent transfusions. However, when the investigators found that the haemoglobin concentrations in the two groups showed little difference they decided to stop transfusions and observe patients closely. They found that stopping blood transfusions had no detrimental effect.
Professor Sir David Weatherall states: “Our findings suggest that haemoglobin E â thalassaemia can be managed without transfusion in many patients, even with low haemoglobin levels.”