Polycythemia vera: stem-cell and probable clonal origin of the disease.
摘要：
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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关键词：
Blood Platelets Erythrocytes Granulocytes Hematopoietic Stem Cells Humans Polycythemia Vera Glucosephosphate Dehydrogenase Heterozygote Phenotype Female
DOI：
10.1056/NEJM197610212951702
被引量：
年份：
1976
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