Quantitative evaluation of the mechanisms of the anaemia in heterozygous beta-thalassaemia.
Ferrokinetic studies were carried out in 8 patients with heterozygous beta-thalassaemia with anaemia of varying severity. Effective and ineffective erythropoiesis, mean red cell lifespan and non-erythroid iron turnover were estimated from the experimental data through a mathematical model of iron kinetics. Erythropoietic activity was markedly increased in all patients, but was variably ineffective (from 10 to 74%). A negative correlation (r = 0.855, P less than 0.01) was found between the amount of ineffective erythropoiesis and Hb level. Red cell lifespan was variably shortened and there was a negative correlation between the degree of daily peripheral haemolysis and Hb level (r = 0.733, P less than 0.05). Non-erythroid iron turnover was increased in most patients. The results provide quantitative measurements of the mechanisms responsible for the wide variation of the Hb level in heterozygous beta-thalaeeaemia. Ineffective erythropoiesis seems to be the major reason for the anaemia. Peripheral haemolysis contributes to it, especially in the most severely affected patients. The increased non-erythroid iron turnover may be responsible for the pathology which characterizes heterozygotes in the adult life.
D000293:Adolescent; D000328:Adult; D000740:Anemia / Q000097:blood; D002648:Child; D004905:Erythrocyte Aging; D004912:Erythrocytes / Q000378:metabolism; D004920:Erythropoiesis; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006461:Hemolysis; D006579:Heterozygote; D006801:Humans; D007501:Iron / Q000097:blood*; D008297:Male; D008875:Middle Aged; D013789:Thalassemia / Q000097:blood*
D006454:Hemoglobins; D007501:Iron