Glucose-6-phosphate dehydrogenase deficiency, glutathione levels and other haematological indices among individuals with sickle cell disorders

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The study involved 310 sickle cell patients with 100 control subjects, and it was aimed at investigating whether the presence of G6PD deficiency among individuals with sickle cell disorders would have any significant impact on some haernatological indices and the clinical state. Blood samples were taken from clinically stable patients at the Sickle Cell Clinic of KATH, Kumasi, where previous studies had shown a high frequency of G6PD deficiency among the sicklers. Their average age was 10 years. The percentage of sicklers who had experienced two or more crises for the previous year in G6PD-normal sicklers was 51.72 whereas that of G6PD-deficient sicklers was 35.00. A third of each of both groups of sicklers had been haemotransfused before. However, the frequency of haemotransfusion was slightly higher in G6PD-normal sicklers (32.07%) than G6PD-deficient sicklers (30.00%). The above observations confirm the report that sicklers with G6PD-deficiency do not compromise on their combined defects. With the exception of the glutathione (GSH) levels and the reticulocyte count which were in the same range as the control subjects (normal children), all the other haematological indices like GSH stability, haemoglobin (Hb). packed cell volume (PCV), red blood cell count (RBC) and erythrocyte sedimentation rate (ESR) were outside the reference range. The normal trend of the reticulocyte count is an indication that their bone marrow was functioning well and more importantly, the patients were not anaemic at the period of study. The association of low levels of GSH among G6PD-deficient sicklers could not be established. This is consistent with the tindings that there is no direct relationship between G6PD deficiency and sickle cell disease. Statistically, there was no significant difference (p> 0.05) between G6PD-normal and G6PD-deficient sicklers in the determined haematological indices except the glutathione stability. However, there was significant difference in GSR stability which was much expected since the stability test is one of the recommended models for screening G6PD deficiency among subjects. Apart from the reticulocyte count, the presence of the sickling condition among the patients had a significant impact (p< 0.001) on their .haematological indices. This also confirms the fact that the presence of the sickling Hb affects the survival time of red cells as well as the flow properties of the blood. A statistically significant negative c9rrelatjon was found between GSH levels, and Hb, PC’v, and RB( in each group of sicklers. This indicates that those with anaemia have higher glutathione levels than non-anaemic individuals. There was however a statistically significant positive correlation between GSH levels, and reticulocyte count and ESR in the sicklers which confirms the high reticulocyte count and ESR associated with anaemia. A similar trend was recorded for the normal children, i.e. those without any of the defects. The correlation between the GSH level, and Hb, PCV, RBC, reticulocyte count and ESR was strong (r: ± 0.60 to 0.79) in all the subjects under study. The coefficients of determination (r2,,) between the GSH levels, and other indices including Rb, PCV, RBC, reticulocyte count and ESR in the subjects were approximately equal to 0.5 or 0.6. This shows that the variability of the GSH levels can be used to predict the change in Hb, PCV, RBC, reticulocyte count and ESR in all the subjects under study by at least 50% (r2:0.5).
A thesis submitted to the School of Graduate Studies in partial fulfilment of the requirements for the degree of Master of Philosophy in Biochemistry, 2004