Abstract

Background: As to whether the presence of Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency ameliorates or worsens sickle cell disease (SCD) is still not clear. Aim and Objectives: This study was therefore aimed at investigating whether the presence of G6PD deficiency among SCD subjects (HbSS and HbSC) would have any significant impact on the severity of crisis and haematological indices. Subjects and Methods: A structured questionnaire was used to obtain information on the clinical state of the subjects. The qualitative methaemoglobin reduction method was used to screen for G6PD deficiency, followed by the measurement of some haematological indices. Results: The data showed that the different genotypes of SCD subjects had similar clinical features, voiding of darkened urine, frequency of crises and blood transfusion, p > 0.05. Statistically, the presence of G6PD-deficency among the HbSS and HbSC patients had no significant impact on their haematological indices (p > 0.05), except the glutathione stability, (p < 0.05). However, a statistically significant difference occurred between the haematological indices of the SS and SC patients (p < 0.05), with improved values in the SC subjects. The presence of the G6PD in the SCD population was quite low (6.49%) compared with other similar studies (8 - 30 %) among the general population. These findings strongly support some reports that SCD subjects with G6PD-deficiency do not show a compromised function due to the combined defects.

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