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

Abstract

Abstract. 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 hemeheme interactions were impaired. At 370C 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.

Erythrocyte Hb-S Concentration AN IMPORTANT FACTOR IN THE LOW OXYGEN AFFINITY OF BLOOD IN SICKLE CELL ANEMIA

“…In hypoplastic anemias it seems clear that the 2.3-DPGinduced shift in the 02-Hb equilibrium curve may compensate for up to half the oxygen deficit implicit in the anemia (19). Some hemoglobinopathies may be associated with altered oxygen affinity because of the nature of the substitution in the hemoglobin molecule (20)(21)(22) or because the substitution involves one of the binding sites for 2,3-DPG (23). The higher oxygen affinity of cord blood has recently been explained by the reduced interaction between fetal hemoglobin (Hb F) and 2,3-DPG (24,25).…”

Hemoglobin Brigham (α2Aβ2100 Pro→Leu). HEMOGLOBIN VARIANT ASSOCIATED WITH FAMILIAL ERYTHROCYTOSIS

“…At least 10 different variants have been reported (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). With the exception of Hb Olympia (12), these abnormal hemoglobins can be separated from Hb A by electrophoresis or chromatography.…”

Hemoglobin Yoshizuka (G10(108)β asparagine→aspartic acid): a new variant with a reduced oxygen affinity from a Japanese family

“…These changes are due to the replacement of asparagine by threonine near the contact ax-fl2 (10). The discovery of other variants, hemoglobins Chesapeake (4), J Cape Town (6), Yakima (7,8), and Kempsey (9) has strengthened the importance of the G (Table II). Rosemeyer and Huehns (40) have pointed out that the binding of oxygen contributes to the tendency of hemoglobin to dissociate.…”