| J Clin Invest. 1969 August; 48(8): 1376–1386. doi: 10.1172/JCI106103. | PMCID: PMC322364 |
Erythrocytosis associated with hemoglobin Rainier: oxygen equilibria and marrow regulation John W. Adamson, Julian T. Parer, George Stamatoyannopoulos, and S. Heinenberg Division of Hematology, Department of Medicine, University of Washington School of Medicine, Seattle, Washington 98105 Division of Medical Genetics, Department of Medicine, University of Washington School of Medicine, Seattle, Washington 98105 Department of Obstetrics and Gynecology, University of Washington School of Medicine, Seattle, Washington 98105 Abstract Hemoglobin Rainier (β 145 tyrosine→histidine) is an abnormal hemoglobin associated with increased oxygen affinity, decreased heme-heme interaction, presence of a Bohr effect, and erythrocytosis, but without obvious clinical sequelae. Regulation of erythropoiesis was studied in affected members of families having either hemoglobin Rainier or Yakima, abnormal hemoglobins associated with erythrocytosis. Apart from the elevated but stable hemoglobin concentration and red cell mass, parameters of red cell production in the subjects were normal. Initially normal values of erythropoietin excretion were increased by phlebotomy indicating a significant hypoxic stress at an otherwise normal hematocrit. This stress led to increased reticulocyte production and an eventual return to the prephlebotomy hematocrit. The erythrocytosis in carriers of hemoglobins Rainer and Yakima appears to be secondary to the increased oxygen affinity and this, with the response to phlebotomy, is consistent with the postulate that the renal sensor tissue regulating erythropoietin production is primarily influenced by the oxygen tensions of venous rather than arterial blood. Full text Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (1.8M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References. Images in this article Click on the image to see a larger version. These references are in PubMed. This may not be the complete list of references from this article. - Gordon, AS; Cooper, GW; Zanjani, ED. The kidney and erythropoiesis. Semin Hematol. 1967 Oct;4(4):337–358. [PubMed]
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