Interaction between iron deficiency and alpha-thalassaemia: the in vitro effect of haemin on alpha-chain synthesis.
Two conditions are liable to lower the alpha:beta globin biosynthesis ratio in reticulocytes: iron deficiency and alpha-thalassaemia. The present paper studies the effect of haemin on reticulocytes from 12 patients who have alpha-thalassaemia and/or are iron deficient. The alpha:beta globin biosynthesis ratio was improved in all these cases. 4 showed initially an alpha:beta synthesis ratio usually associated with alpha-thalassaemia type-1; on the addition of haemin the ratio rose to that associated with alpha-thalassaemia type-2. In the other 8 patients the ratio was initially typical for alpha-thalassaemia type-2, and on addition of haemin the ratio became normal. It is suggested that in iron deficient patients a diagnosis of alpha-thalassaemia type-1 or type-2 cannot be made unless haemin has been added to the test system. If this is not done iron deficiency alone can cause the alpha:beta globin synthesis ratio to resemble that associated with alpha-thalassaemia type-2, and iron deficiency in combination with alpha-thalassaemia type-2 can cause the ratio to resemble that typical for alpha-thalassaemia type-1. Reticulocytes from 8 alpha-thalassaemic patients without iron deficiency did not show a marked haemin effect (less than 5%), and in 1 patient with iron overload, the ratio actually fell by about 10%.
D000293:Adolescent; D000328:Adult; D000747:Anemia, Hypochromic / Q000097:blood / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D005914:Globins / Q000096:biosynthesis*; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000494:pharmacology*; D006801:Humans; D066298:In Vitro Techniques; D007223:Infant; D000090463:Iron Deficiencies; D008297:Male; D008875:Middle Aged; D013789:Thalassemia / Q000097:blood* / Q000378:metabolism
D006418:Heme; D006427:Hemin; D005914:Globins