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Research Article Free access | 10.1172/JCI106103

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

Find articles by Adamson, J. in: PubMed | Google Scholar

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

Find articles by Parer, J. in: PubMed | Google Scholar

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

Find articles by Stamatoyannopoulos, G. in: PubMed | Google Scholar

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

Find articles by Heinenberg, S. in: PubMed | Google Scholar

Published August 1, 1969 - More info

Published in Volume 48, Issue 8 on August 1, 1969
J Clin Invest. 1969;48(8):1376–1386. https://doi.org/10.1172/JCI106103.
© 1969 The American Society for Clinical Investigation
Published August 1, 1969 - Version history
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Abstract

Hemoglobin Rainier (β145 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.

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