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Polycythemia Vera: Stem-Cell and Probable Clonal Origin of the Disease
Abstract
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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Citations
Cited by
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A Gain-of-Function Mutation of JAK2 in Myeloproliferative Disorders
Kralovics, R., Passamonti, F., Buser, A. S., Teo, S.-S., Tiedt, R., Passweg, J. R., Tichelli, A., Cazzola, M., & Skoda, R. C. (2005) · New England Journal of Medicine · doi:10.1056/nejmoa051113 · Cited by 3,470
Levine, R. L., Wadleigh, M., Cools, J., Ebert, B. L., Wernig, G., Huntly, B. J. P., Boggon, T. J., Wlodarska, I., Clark, J. J., Moore, S., et al. (2005) · Cancer Cell · doi:10.1016/j.ccr.2005.03.023 · Cited by 2,993
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Ghoreschi, K., Laurence, A., & O’Shea, J. J. (2009) · Immunological Reviews · doi:10.1111/j.1600-065x.2008.00754.x · Cited by 1,149
Metadata
- Authors
- Adamson, J. W., Fialkow, P. J., Murphy, S., Prchal, J. F., & Steinmann, L.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1976-10-21
- Volume
- 295
- Issue
- 17
- Pages
- 913-916
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2038611905
- Cited by
- 640
- References
- 0
- FWCI
- 17.43
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Polycythemia Vera: Stem-Cell and Probable Clonal Origin of the Disease. https://app.cochat.ai/papers/10.1056/nejm197610212951702
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- Adamson, J. W., Fialkow, P. J., Murphy, S., Prchal, J. F., & Steinmann, L. (1976). Polycythemia Vera: Stem-Cell and Probable Clonal Origin of the Disease. New England Journal of Medicine. https://doi.org/10.1056/nejm197610212951702
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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