Beta thalassaemia in Papua New Guinea

The treatment of homozygous beta thalassaemia in Papua New Guinea during the period 1980-85 is reviewed. The results showed that the average pre-transfusion haemoglobin was 4.5 g/dl. Evidence of hypersplenism, based on spleen size or annual blood requirement, was present in all patients by age 3-4 years. Mean age of death was less than 9 years. These results are compared with previously published results of therapies from countries with more intensive treatment regimes. It is concluded that supertransfusion and hypertransfusion regimes both offer significant improvements, in duration and quality of life, upon the currently followed protocol.