Abstract:
Background: Previously published data have demonstrated that sickle red blood cells produce twice as much
reactive oxygen species (ROS) suggesting that co-inheritance of sickle cell disease (SCD) and glucose 6-phosphate
dehydrogenase (G6PD) enzymopathy could lead to more severe anaemia during sickling crises. Elevated foetal
haemoglobin (Hb F) levels have been shown to have positive modulatory effects on sickling crises and disease
outcomes. This study sought to assess how inheritance of G6PD enzymopathy affects the level of Hb F and
haemoglobin concentration in adults in steady state.
Methods: This cross-sectional study selected 100 out-patients (41 males and 59 females) visiting the University of
Cape Coast hospital, between January, 2016 and May, 2016. Cellulose acetate electrophoresis (pH 8.2–8.6),
methaemoglobin reductase test, modified Betke alkaline denaturation methods were used to investigate
haemoglobin variants, qualitative G6PD status, and %Hb F levels in venous blood samples drawn from these
participants. Data was analysed with GraphPad Prism 6 and SPSS and significance set at p < 0.05.
Results: Forty one percent of the participants demonstrated qualitative G6PD enzymopathy whereas only 10%
demonstrated Hb AS type (Sickle cell trait, SCT). 5% of the participants co-inherited SCT and G6PD enzymopathy.
%Hb F levels in G6PD deficient males was significantly higher than in G6PD deficient females [(p = 0.0003, 2.696%
(males) vs 1.975% (females)], although the %Hb F levels was comparable in non-G6PD deficient individuals. %Hb F
levels were significantly elevated in males with SCT only (p < 0.05), or G6PD enzymopathy only (p < 0.0001), or
SCT + G6PD enzymopathy (p < 0.0001) compared to males with none of these pathologies even though their
respective haemoglobin levels were comparable. Male participants with G6PD enzymopathy + SCT co-inheritance
had significantly elevated %Hb F when compared to their counterparts with only G6PD enzymopathy (p < 0.001).
Male gender [(p = 0.001, OR: 6.912 (2.277–20.984)] partial defective G6PD enzyme [(p = 0.00, OR: 7.567E8 (8.443E7–6.
782E9)] SCT [(p = 0.026, OR: 4.625 (1.196–17.881)] were factors associated with raised %Hb F levels ≥2.5.
Conclusion: The inheritance of G6PD defect and/or SCT significantly elevate %Hb F levels in the steady state even
though haemoglobin levels are not affected.
