Blood transfusion in beta thalassaemia major

Abstract

Conventional treatment of beta thalassaemia major is based on regular blood transfusion from early childhood. Maximum effectiveness of transfusion therapy depends on the following. (1) Availability of safe blood. Donation programmes should aim at retaining repeat donors, who carry decreased risk of transmitting blood-borne infections. Donors should be screened with laboratory tests performed to the highest possible standard of quality. Selection of safe donors can be improved by the adoption of questionnaires containing direct questions on risk factors for transfusion transmissible infections. (2) Use of good quality red blood cells, which should be leucodepleted, preferably by filtration, that can be carried out at the bedside. (3) Regular evaluation of blood transfusion indices, including mean level of haemoglobin maintained, annual blood requirement, daily haemoglobin fall, mean transfusion interval, transfusion reaction rate. This can be assisted by the use of a computerized patient record. (4) Maintenance of a permanent record of the patient's blood group genotype (including at least Rh, Kell, Kidd and Duffy systems) and any red cell antibodies that develop. This is mandatory to ensure optimal survival of transfused red cells. (5) Continuous monitoring of transfusion transmissible infections. (6) Vaccination against hepatitis B of all suitable patients. (7) Intensive iron chelation. This should be done by regular subcutaneous administration of desferrioxamine B. Oral chelators, which are currently under laboratory and clinical evaluation, are not yet available for general use.