Abstract
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.
Publication types
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adult
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Aspartic Acid / analysis
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Blood Protein Electrophoresis
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Chromatography
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Female
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Hemoglobinopathies / complications*
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Hemoglobinopathies / genetics
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Hemoglobins, Abnormal* / analysis
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Hemoglobins, Abnormal* / metabolism
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Humans
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Oxygen / metabolism
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Polycythemia Vera / blood*
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Polycythemia Vera / etiology
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Tyrosine / analysis
Substances
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Hemoglobins, Abnormal
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Aspartic Acid
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Tyrosine
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Oxygen