Impact of Foetal Haemoglobin on Disease Severity among Sickle Cell Subjects

Abstract

Objective: Haemolysis is a common complication of Sickle cell disease, exhibiting considerable variability among affected individuals. Established haemolysis markers including lactate dehydrogenase activity (LDH) and bilirubin have been reported as reliable indicators of disease severity. Foetal haemoglobin plays a pivotal role in Sickle Cell Disease by neutralizing Haemoglobin-S (HbS) polymerization, consequently extending the lifespan of red blood cells and influencing overall clinical outcomes. This study aims to investigate the impact of foetal haemoglobin on disease severity among sickle cell subjects. Method: This study involved forty (40) sickle cell subjects and thirty (30) normal subjects. Blood samples from the participants were analysed for lactate dehydrogenase activity, bilirubin concentration, and foetal haemoglobin concentration. The alkali denaturation technique was employed for HbF analysis, while diagnostic kits were used for LDH and bilirubin assays. Results: Foetal haemoglobin concentrations below 10% have been defined as low. The HbF concentration of the sickle cell subjects was relatively low (5.23%) but significantly higher than the normal subjects (1.84%). Similarly, there was a significant elevation in markers of haemolysis in the sickle cell subjects compared to the normal subjects. Subjects with HbF>10% demonstrated a noteworthy increase in haemolysis markers compared to those with HbF<5%. Additionally, a significant inverse relationship was observed between HbF concentration and haemolysis markers. Conclusion: The study highlights a significant inverse correlation between foetal haemoglobin concentration and disease severity among sickle cell subjects and also contributes valuable insights to our understanding of sickle cell disease pathology.