Polycythemia vera. The in vitro response of normal and abnormal stem cell lines to erythropoietin.
Bone marrow cells from two glucose-6-phosphate dehydrogenase (G-6-PD) heterozygotes with polycythemia vera were cultured to determine whether progenitors which wre not of the polycythemia vera clone were present, and, if present, which cell lines contributed to the increase in erythroid colonies observed in response to added erythropoietin (ESF). To accomplish this, the G-6-PD isoenzyme activity of individual erythroid colonies was determined. All of the erythroid colonies analyzed in cultures without added ESF, contained the G-6-PD isoenzyme type characteristic of the abnormal clone. With higher ESF concentrations in the culture, however, there was an increase in the colonies that were not of the polycythemia vera clone. Analysis of the ratio of the various types of colonies indicated that normal and polycythemia vera cells are capable of responding to ESF in vitro. In selected patients, this technique permits analysis of the ratios of normal to abnormal cells during the course of the disease, in response to therapy and during late complications, such as myelofibrosis or leukemic transformation.
D002999:Clone Cells / Q000201:enzymology; D004912:Erythrocytes / Q000201:enzymology; D004920:Erythropoiesis; D004921:Erythropoietin / Q000494:pharmacology; D005954:Glucosephosphate Dehydrogenase / Q000097:blood; D006098:Granulocytes / Q000201:enzymology; D006412:Hematopoietic Stem Cells / Q000201:enzymology; D006801:Humans; D007527:Isoenzymes / Q000097:blood; D011087:Polycythemia Vera / Q000473:pathology / Q000503:physiopathology*
D007527:Isoenzymes; D004921:Erythropoietin; D005954:Glucosephosphate Dehydrogenase