Quantitative Evaluation of the Mechanisms of the Anaemia in Heterozygous β‐Thalassaemia

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Abstract
Ferrokinetic studies were carried out in 8 patients with heterozygous .beta.-thalassemia and anemia of varying severity. Effective and ineffective erythropoiesis, mean red cell [RBC] lifespan and non-erythroid Fe turnover were estimated from experimental data through a mathematical model of Fe kinetics. Erythropoietic activity markedly increased in all patients, but was variably ineffective (from 10-74%). Negative correlation (r [correlation coefficient] = - 0.855, P < 0.01) was found between amount of ineffective erythropoiesis and Hb level. RBC lifespan was variably shortened and there was negative correlation between degree of daily peripheral hemolysis and Hb level (r = - 0.733, P < 0.05). Non-erythroid Fe turnover increased in most patients. The results provide quantitative measurements of mechanisms responsible for wide Hb level variation in heterozygous .beta.-thalassemia. Ineffective erythropoiesis seems to be the major reason for the anemia. Peripheral hemolysis contributes to it, especially in the most severely affected patients. Increased non-erythroid Fe turnover may be responsible for pathology which characterizes heterozygotes in the adult life.