Quantitation of ineffective erythropoiesis from the incorporation of [15N] delta-aminolaevulinic acid and [15N] glycin into early labelled bilirubin. II. Anaemic patients.
The incorporation of [15N]delta-aminolaevulinic acid and [15N glycine into haemoglobin haem and early labelled bilirubin was measured in subjects with various haematological disorders. The clearance of [14C bilirubin was used to measure bilirubin production rate, and the magnitude of the various sources of bilirubin production and the percentage ineffective erythropoiesis were calculated. Ineffective erythropoiesis was found to be a major factor in the production of the anaemia in patients with the following disorders: megaloblastic anaemia associated with the Lesch-Nyhan syndrome, thalassaemia intermedia, sideroblastic anaemia, and the anaemia of chronic disorders. In three patients with iron-deficiency anaemia ineffective erythropoiesis was increased, but was of minor importance in the production of the anaemia, while in two patients with aplastic anaemia and one with macrocytosis of alcoholism there was no increase in ineffective erythropoiesis.
D000293:Adolescent; D000368:Aged; D000622:Aminolevulinic Acid / Q000378:metabolism*; D000740:Anemia / Q000378:metabolism*; D000741:Anemia, Aplastic / Q000378:metabolism; D000747:Anemia, Hypochromic / Q000378:metabolism; D000756:Anemia, Sideroblastic / Q000378:metabolism; D001663:Bilirubin / Q000096:biosynthesis*; D002648:Child; D004920:Erythropoiesis; D005260:Female; D005998:Glycine / Q000378:metabolism*; D006418:Heme / Q000096:biosynthesis; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000378:metabolism; D007982:Levulinic Acids / Q000378:metabolism*; D008297:Male; D008875:Middle Aged
D007982:Levulinic Acids; D006418:Heme; D000622:Aminolevulinic Acid; D001663:Bilirubin; D005998:Glycine