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Hematological Effects of the Co-Inheritance of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease

Michael H Mezgebe, Nazeer Alli, Aamina Moosa

    Clinical Laboratory
    |August 8, 2026
    PubMed

    Insights

    Co-inheritance of sickle cell disease (SCD) and glucose-6-phosphate dehydrogenase deficiency (G6PDD) in HbAS individuals does not cause anemia but indicates increased hemolysis. This suggests potential complications from chronic hemolysis beyond anemia in these patients.

    Area of Science:

    • Hematology
    • Genetic Disorders
    • Clinical Medicine

    Background:

    • Glucose-6-phosphate dehydrogenase deficiency (G6PDD) and sickle cell disease (SCD) are hemolytic anemias.
    • Limited research exists on the hematological impact of SCD and G6PDD co-inheritance, with conflicting findings.

    Purpose of the Study:

    • To compare hematological parameters and hemolysis markers (LDH, UBR) in SCD patients versus those with SCD/G6PDD co-inheritance.

    Main Methods:

    • 214 SCD patients (HbSS and HbAS variants, with/without G6PDD) were studied over 14 years.
    • SCD diagnosed via HPLC; G6PD status by spot testing.
    • Full blood count (FBC) parameters, UBR, and LDH levels were recorded.

    Main Results:

    • No significant differences in FBC or UBR were found between HbSS and HbSS/G6PDD patients.
    • HbAS/G6PDD patients showed significantly higher mean UBR levels (p < 0.05) compared to HbAS alone.
    • FBC parameters did not differ significantly between HbAS and HbAS/G6PDD groups.

    Conclusions:

    • G6PDD co-inheritance with HbAS does not lead to anemia.
    • Elevated UBR in HbAS/G6PDD patients suggests ongoing hemolysis.
    • Chronic hemolysis in these patients may lead to complications beyond anemia.
    Abstract

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