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Hemoglobin Yakima: II. High Blood Oxygen Affinity Associated with Compensatory Erythrocytosis and Normal Hemodynamics*

Miles J. Novy, Miles J. Edwards and James Metcalfe§

Heart Research Laboratory and Division of Chest Diseases, Department of Medicine, University of Oregon Medical School, Portland, Oregon

Research Associate, National Institute of Child Health and Human Development. Present address: Dept. of Surgery, Boston University School of Medicine, Boston, Mass.

§ Professor of Medicine, Oregon Heart Association Chair of Cardiovascular Research. Address requests for reprints to Dr. James Metcalfe, Heart Research Laboratory, University of Oregon Medical School, Portland, Oreg. 97201.

* Received for publication 16 May 1967 and in revised form 23 June 1967.

Published November 1967

Erythrocytosis without clinical illness was noted in a man and his two daughters. Their blood contained approximately 62% hemoglobin A and 38% a new hemoglobin, designated hemoglobin Yakima. The oxygen affinity of whole blood from each subject was greatly increased and heme-heme interactions were impaired. At 37°C and a plasma pH of 7.40, the oxygen pressure required to produce 50% saturation of hemoglobin with oxygen was only 12 mm Hg as compared with a normal of 26 mm Hg. The high oxygen affinity of this blood is attributed to the presence of hemoglobin Yakima; and the increased oxygen affinity was shown to be characteristic of the isolated abnormal hemoglobin. A Bohr effect was present in hemoglobin Yakima.

Arterial oxygen pressure, oxygen consumption, and cardiac output at rest were normal. With respect to oxygen delivery to tissues, the increased hemoglobin concentration appears to be the major compensation for the marked displacement of the oxygen-hemoglobin equilibrium curve, although other factors may contribute. The finding of high normal quantities of erythropoietin in the urine is consistent with this degree of erythrocytosis.

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