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An increased Bohr effect in sickle cell anemia.

Y Ueda, R L Nagel, R M Bookchin

    Blood
    |March 1, 1979
    PubMed
    Summary

    Sickle cell anemia (SS) red blood cells exhibit an exaggerated Bohr effect, releasing more oxygen at lower pH levels. This heightened response, particularly in tissue capillaries, increases the risk of sickling and may pose hazards during acidosis.

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    Area of Science:

    • Hematology
    • Physiology
    • Biochemistry

    Background:

    • Hemoglobin S (HbS) gelation and red blood cell sickling are pH-dependent.
    • HbS influences red blood cell oxygen affinity.
    • These factors suggested an abnormal Bohr effect in sickle cell anemia.

    Purpose of the Study:

    • To investigate the effects of pH variation on the in vitro oxygen affinity of whole blood from individuals with sickle cell anemia (SS) compared to normal donors.
    • To quantify the Bohr effect in SS blood.

    Main Methods:

    • Studied the in vitro oxygen affinity of whole blood from SS patients and normal donors.
    • Varied blood pH while maintaining a constant carbon dioxide tension (40 mm Hg) at 37°C.

    Main Results:

    • The Bohr effect in SS blood was significantly increased between pH 7.4 and 7.2 (blood) / 7.2 and 7.0 (cell).
    • The magnitude of the Bohr effect in SS blood (delta log p50/delta pH = -0.92 to -0.99) was more than double that of normal blood ( -0.42 to -0.46).
    • A drop in SS blood pH below 7.4 in tissue capillaries leads to a greater decrease in oxygen affinity and substantial oxygen release.

    Conclusions:

    • Sickle cell anemia red blood cells display an amplified Bohr effect, leading to excessive oxygen release under acidic conditions.
    • Even mild acidosis can be dangerous for patients with sickle cell disorders due to increased sickling risk.
    • Understanding the abnormal Bohr effect is crucial for managing sickle cell disease complications.

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