Binding of serum ferritin to concanavalin A: patients with homozygous beta thalassaemia and transfusional iron overload.
Serum ferritin concentrations have been measured in 124 patients with homozygous beta thalassaemia who were between 2 and 21 years old, had received 11--504 units of blood but had not undergone splenectomy. There were highly significant correlations between serum ferritin concentration and both the amount of blood transfused and alanine amino-transferase (ALT) activity. However, multivariate analysis showed that units of blood and ALT activity together only accounted for about 30% of the variation in serum ferritin concentration. Little of the remaining variation could be explained by other variables related to iron metabolism or liver damage. The concentration of concanavalin A binding ferritin increased rapidly with the number of units of blood up to 100 units but thereafter showed no further increase with number of transfusions. The concentration of non-binding ferritin was more closely related to transfusion load. These results suggest that the secretion of glycosylated ferritin from reticuloendothelial cells reaches a maximum with increasing iron accumulation, perhaps reflecting a maximum rate of synthesis. Ferritinaemia in patients with transfusional iron overload therefore seems to be the result of the combined effects of increased ferritin synthesis and the release of intracellular ferritin from damaged cells. A simple relationship between serum ferritin and iron stores cannot be assumed when ferritin concentrations exceed 4000 microgram/l or in patients who have received more than 100 units of transfused blood.
D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003208:Concanavalin A; D005293:Ferritins / Q000097:blood*; D006720:Homozygote; D006801:Humans; D007501:Iron / Q000097:blood*; D013789:Thalassemia / Q000097:blood* / Q000628:therapy; D065227:Transfusion Reaction
D003208:Concanavalin A; D005293:Ferritins; D007501:Iron; D000410:Alanine Transaminase