Polycythemia produced by hemoglobin Osler (beta-145 (HC2) Tyr yields Asp)

Polycythemia in a black woman was associated with hemoglobin Osler. In this hemoglobin the tyrosine at the penultimate position of the beta chain is replaced by aspartic acid. As a result oxygen affinity of the hemoglobin is very high, heme-heme interaction is absent, and the influence of hydrogen ion and 2,3 diphosphoglycerate on oxygen affinity is attenuated.