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Assessment of Iron Loading in Cypriot Homozygous Beta-Thalassaemic Children

Forty-one patients with homozygous 6-thalassaemia and three with haemoglobin H disease were examined for liver iron concentration, serum ferritin level and the presence of extra medullary haemopoeisis (EMU). Significant correlations were obtained between transfused iron load versus age, liver iron concentration versus transfused iron load and age versus liver iron concentration. Liver iron concentration and age distinguish between severe and intermediate cases. Correlation between serum ferritin and liver iron concentration is highly significant in patients without EMU. Those patients with histological evidence of EMU failed to show this correlation. It is postulated that the presence of EMU is a reflection of ineffective erythropoeisis in the bone marrow of undertransfused patients and that there is thus a large catabolic contribution to the total serum ferritin, explaining the poor correlation between the serum ferritin and liver iron concentration in this group.