Ferrokinetic and erythrokinetic studies in alpha and beta thalassaemia

Ferrokinetic and erythrokinetic studies were performed in 25 non-splenectomized patients with alpha or beta thalassaemia. Nine of these had HbH disease and six had HbE/beta thalassaemia or homozygous beta thalassaemia. In HbH disease, a mild anaemia was associated with severe peripheral haemolysis, increased splenic sequestration and only a moderate degree of ineffective erythropoiesis. By contrast, in the beta thalassaemia syndromes, a more marked anaemia was associated with prominent ineffective erythropoiesis and mild peripheral haemolysis. These findings indicate that the pathogenesis of anaemia in alpha and beta thalassaemia is different, haemolysis dominating in HbH disease and ineffective erythropoiesis in HbE/beta thalassaemia and homozygous beta thalassaemia.