Reexpression of normal stem cells in erythroleukemia during remission.
A patient with erythroleukemia and heterozygous for the Mediterranean variant of the X-linked enzyme glucose-6-phosphate dehydrogenase (G6PD) was studied to determine the number and type of progenitor cells in which the disease arose. G6PD mosaicism was assessed by the different rate of utilization of 2-deoxy-glucose-6-phosphate (2dG6P) by normal and Mediterranean variants of G6PD. Erythroleukemia is established as a clonal disease involving a precursor cell common to the erythroid and myeloid lines. After intensive chemotherapy, restoration of nonmonoclonal hemopoiesis is achieved, as indicated by the reappearance of the mosaic phenotype in hemopoietic cell populations.
D000368:Aged; D002455:Cell Division; D002999:Clone Cells; D004912:Erythrocytes / Q000201:enzymology; D005260:Female; D014644:Genetic Variation; D019298:Glucose-6-Phosphate / Q000031:analogs & derivatives*; D005954:Glucosephosphate Dehydrogenase / Q000235:genetics* / Q000378:metabolism; D005958:Glucosephosphates / Q000378:metabolism; D006098:Granulocytes / Q000201:enzymology; D006410:Hematopoiesis; D006412:Hematopoietic Stem Cells / Q000201:enzymology*; D006579:Heterozygote; D006801:Humans; D004915:Leukemia, Erythroblastic, Acute / Q000097:blood* / Q000201:enzymology; D009030:Mosaicism
D005958:Glucosephosphates; C015785:2-deoxyglucose-6-phosphate; D019298:Glucose-6-Phosphate; D005954:Glucosephosphate Dehydrogenase