Hemoglobin biosynthesis in juvenile erythroleukemia: evidence of imbalanced globin chain synthesis.
In three young patients with erythroleukemia in whom a partial reversion to the fetal pattern of erythropoiesis occurred there was found additionally an imbalance of globin chain synthesis. The synthesis of beta- plus gamma-chains exceeded that of the alpha-chains. In contrast, physiologic hemoglobin F production occurring in newborn infants and increased hemoglobin F production due to rapidly regenerating erythropoiesis in hereditary spherocytosis and after acute erythroblastopenia are characterized by a well balanced globin chain synthesis. These studies indicate that in distinct cases of juvenile erythroleukemia the genuine reversion to fetal erythropoiesis may be associated not only with a depression of hemoglobin synthesis but also with an imbalanced globin chain synthesis. Unlike adult cases of erythroleukemia without reversion to fetal erythropoiesis the imbalance of globin chain synthesis seems to be a more generalized phenomenon in these cases of juvenile erythroleukemia which is not confined to a particular red cell population.
D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D004920:Erythropoiesis; D006454:Hemoglobins / Q000096:biosynthesis*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D004915:Leukemia, Erythroblastic, Acute / Q000378:metabolism*; D008297:Male
D006454:Hemoglobins