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Hemoglobin E, an oxidatively unstable mutation.

H Frischer, J Bowman

    The Journal of Laboratory and Clinical Medicine
    |April 1, 1975
    PubMed
    Summary
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    A new test detected gross turbidity in blood samples, primarily linked to hemoglobin E, an unstable variant. This finding aids in identifying oxidative hemolysis susceptibility and differentiating hemoglobin E from other types.

    Area of Science:

    • Hematology
    • Biochemistry
    • Genetics

    Background:

    • Erythrocytic enzyme deficiencies can lead to various health issues.
    • Hemoglobin variants, such as hemoglobin E, are prevalent in certain populations.
    • Oxidative stress plays a role in red blood cell disorders.

    Purpose of the Study:

    • To evaluate a new diagnostic test for erythrocytic enzyme deficiencies.
    • To investigate the association between observed turbidity and specific hemoglobin types.
    • To characterize the oxidative instability of hemoglobin E.

    Main Methods:

    • A novel assay utilizing 2,6-dichlorophenolindophenol reduction by glutathione was employed.
    • Blood samples from 3,159 individuals across diverse geographical locations were analyzed.

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  • Hemolysates were incubated with dichlorophenolindophenol to assess hemoglobin precipitation rates.
  • Main Results:

    • Gross turbidity was observed in 81 samples, predominantly from Southeast Asia.
    • 78 of the turbid samples contained hemoglobin E (Hb EE or Hb AE).
    • Precipitation rates showed Hb EE > Hb AE > other hemoglobins, indicating Hb E's oxidative instability.

    Conclusions:

    • The new test effectively detects abnormalities associated with hemoglobin E.
    • Hemoglobin E is an oxidatively unstable hemoglobin, increasing susceptibility to hemolysis.
    • This method allows for simple detection and differentiation of Hb E from Hb C and Hb O-Arabia.