Fetal erythropoiesis in juvenile chronic myelocytic leukemia.
Red cell enzymes, 2,3-diphosphoglycerate (2,3-DPG) and adenosine triphosphate (ATP), were evaluated in a 23-mo-old boy with juvenile chronic myelocytic leukemia (JCML) at the onset of his illness and 6 mo later during the accelerated phase. The activities of the age-dependent red cell enzymes, hexokinase, aldolase, pyruvate kinase, and glucose-6-phosphate dehydrogenase, were elevated, as were the concentrations of red cell 2,3-DPG and ATP, consistent with a young red cell population metabolizing at an increased glycolytic rate. The activities of the non-age-dependent enzymes, glyceraldehyde-3-phosphate dehydrogenase (G3PD), phosphoglycerate kinase, and enolase, were also increased to levels similar to or greater than those observed in term infants. As the illness progressed, the activity of red cell G3PD increased further, and phosphoglucose isomerase activity increased markedly. These results are consistent with the prior suggestion that JCML represents a reversion to "fetal" erythropoiesis.
D002675:Child, Preschool; D004912:Erythrocytes / Q000201:enzymology; D004920:Erythropoiesis; D005312:Fetal Blood; D005634:Fructose-Bisphosphate Aldolase / Q000097:blood; D005956:Glucose-6-Phosphate Isomerase / Q000097:blood; D005954:Glucosephosphate Dehydrogenase / Q000097:blood; D005987:Glyceraldehyde-3-Phosphate Dehydrogenases / Q000097:blood; D006593:Hexokinase / Q000097:blood; D006801:Humans; D007951:Leukemia, Myeloid / Q000097:blood / Q000503:physiopathology*; D008297:Male; D010732:Phosphofructokinase-1 / Q000097:blood; D010735:Phosphoglycerate Kinase / Q000097:blood; D010751:Phosphopyruvate Hydratase / Q000097:blood; D011770:Pyruvate Kinase / Q000097:blood
D005954:Glucosephosphate Dehydrogenase; D005987:Glyceraldehyde-3-Phosphate Dehydrogenases; D006593:Hexokinase; D010732:Phosphofructokinase-1; D011770:Pyruvate Kinase; D010735:Phosphoglycerate Kinase; D005634:Fructose-Bisphosphate Aldolase; D010751:Phosphopyruvate Hydratase; D005956:Glucose-6-Phosphate Isomerase