[Anemia 2,3-DPG and tissue oxygenation in beta-thalassemia heterozygotes].
The levels of 2,3-DPG and the value of P50 were determined in unselected, clinically healthy, typical beta-thalassemia heterozygotes and normal controls. Values of 2,3-DPG in the heterozygotes are significantly elevated compared to normals (when expressed per gram of hemoglobin or per volume of red cells) and much higher than their mild hemoglobin deficit would explain; they are elevated even in selected heterozygotes presenting normal hemoglobins levels. These 2,3-DPG values, when expressed per number of erythrocytes, are within normal limits. In parallel, oxygen affinity is lower, as the P50 value is displaced to the right by 2 mmHg above the normal mean, thus assuring adequate tissue oxygenation. The findings suggest that the high 2,3-DPG values of the beta-thalassemia heterozygotes are not determined solely by anemic hypoxia; it is more likely that the microcytic erythrocytosis of heterozygous beta-thalassemia is the cause of this increase. The resulting lower oxygen affinity leads to a decreased stimulation of erythropoiesis and hence to its regulation at lower hemoglobin levels. Accordingly we suggest that the beta-thalassemia trait is a pseudo-anemia, because it is unlikely that these heterozygotes are unable to reach normal hemoglobin values by increasing erythrocyte output.
D019794:2,3-Diphosphoglycerate; D000328:Adult; D000747:Anemia, Hypochromic / Q000097:blood*; D001692:Biological Transport; D004163:Diphosphoglyceric Acids / Q000097:blood*; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006579:Heterozygote; D006801:Humans; D008297:Male; D010100:Oxygen / Q000097:blood; D010101:Oxygen Consumption; D013789:Thalassemia / Q000097:blood*
D004163:Diphosphoglyceric Acids; D006454:Hemoglobins; D019794:2,3-Diphosphoglycerate; D010100:Oxygen
Anémie, 2,3-DPG et oxygénation tissulaire chez les hétérozygotes de beta-thalassémie.