The Effect of Fetal Hemoglobin on RBC Parameters among Sickle Cell Anemia Patients: A Cross Sectional Study from Makkah City; Western Saudi Arabia

Abstract

1.1.Background: Sickle Cell Anemia (SCA) is the most common monogenic disorder that is inherited as an autosomal recessive pattern. Fetal hemoglobin (HbF) plays a major role in ameliorating clinical severity of the SCA where higher expression of HbF is associated with a reduction in the painful episode and thus reduces the number of hospitalization. The current work aimed to investigate the effect of high HbF in SCA adult patients in Makkah city, Western Saudi region who visited Al-Noor Specialist Hospital. 1.2.Methodology:Blood samples in EDTA tubes were collected from 80 SCA adults patients during the period of February, 2015 to April, 2016. Hematological analyses including Complete Blood Cell Count (CBC) and reticulocyte count were performed to assess anemia status. Hemoglobin quantification and separation was performed using High Pressure Liquid Chromatography (HPLC). Patients with hydroxyl urea intake were excluded from the study due to its elevation effect on HbF level. 1.3.Result: Of the 80 studied patients, 60(75%) showed an increased level of HbF above the upper normal limit with a mean of 6.01% +/-3.5. The increase level of HbF was more than 10%, 5-10%, and less than 5% in 6(10%), 24(40%) and 30(50%) patients respectively. A significant association between high HbF and red cell parameters was observed in patients with higher HbF than 10% compared to the other two groups. 1.4.Conclusion:High HbF gives an advantage to the SCA patients where it has an ameliorating effect on the severity and the complications of the disease. The elevated HbF in Makkah city is high among adult SCA patients (75%) but the average expression level is lower than other Saudi regions. This indicates patients from Makkah City have more clinical severity and thus highly attended healthcare is required to minimize disease effect and its complication. Further molecular studies are recommended to find out genetic determinants such as single nucleotide polymorphisms of high HbF expression in SCA.