Polycythemia vera: stem-cell and probable clonal origin of the disease
Two women with polycythemia vera and heterozygosity (GdB/GdA) at the X-chromosome-linked locus for glucose-6-phosphate dehydrogenase were studied to determine the nature of the cellular origin of their polycythemia. In contrast to unaffected tissue, such as skin fibroblasts, which consisted of both...
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Published in | The New England journal of medicine Vol. 295; no. 17; p. 913 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
United States
21.10.1976
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Subjects | |
Online Access | Get more information |
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Summary: | Two women with polycythemia vera and heterozygosity (GdB/GdA) at the X-chromosome-linked locus for glucose-6-phosphate dehydrogenase were studied to determine the nature of the cellular origin of their polycythemia. In contrast to unaffected tissue, such as skin fibroblasts, which consisted of both B and A types, the glucose-6-phosphate dehydrogenase of the patients' erythrocytes, granulocytes and platelets was only of Type A. These results provide direct evidence for the stem-cell nature of polycythemia vera and strongly imply a clonal origin for this disease. The fact that no descendants of the presumed normal stem cells were found in circulation suggests that bone-marrow proliferation in this disorder is influenced by local (intramarrow) regulatory factors. |
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ISSN: | 0028-4793 |
DOI: | 10.1056/NEJM197610212951702 |