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Genetic, cellular and molecular modifiers are responsible for the notoriously variable sickle cell phenotype. Haemoglobin F is a principal modulator of the SCD phenotype. Haemoglobin F inhibits the polymerization of Haemoglobin S and ameliorates the secondary effects of sickling. We measured the Haemoglobin F(HbF) in our population and compared it with other markers associated with clinical severity and clinical status of the patients. Methods: we randomly selected 40 Hemoglobin S(HbS) patients who have never taken hydroxyurea. We measured hemoglobin F levels, packed cell volume (PCV) and reticulocyte count to serve as markers for hemolysis, neutrophils, platelets and MCHC to serve as markers for clinical severity. We searched for a relationship between these laboratory features and frequency of vaso-occlusive crises, transfusion history and number of hospital visits per year. They were compared with twenty healthy Hemoglobin A (HbA) controls. Results: Packed cell volume was significantly lower and the reticulocyte was significantly higher in the hemoglobin S compared with controls. The platelet count of the sickle cell anaemia patients was more than twice the number of the controls. The mean hemoglobin F level was 7.1± 3.5 %. The hemoglobin F was negatively correlated with the platelet count but positively correlated with the total white cell count and haematocrit but there was no significant correlation between hemoglobin F and clinical features Conclusion: The fetal hemoglobin level may not be the only modifier responsible for phenotype in population of hemoglobin S Introduction Sickle cell disease (SCD) is a single gene disorder of red blood cell which presents with chronic hemolysis, vaso-occlusion and end organ damage. (1) This disease predisposes the patients to life-threatening complications, and extensive organ damage resulting in increased morbidity and reduced quality of life. (2) Worldwide, SubSaharan Africa has the highest prevalence of sickle cell anemia. In Nigeria, the prevalence of the sickle cell trait remains steady at 20 to 30%. (3) The homozygous sickle cell anemia affects about 2 to 3% of the Nigerian population. The most recent estimates in Benin City, Nigeria reconfirmed a Sickle cell anaemia prevalence of 2.39% and prevalence of the sickle cell trait of about 23%. (4) The basis of the phenotype of sickle cell anemia is intracellular polymerization of Hemoglobin S upon deoxygenation. This polymerization makes the cells rigid, damaging cell membrane. When the damage to the cell is no longer reversible, the cell assumes a sickle shape. Microvascular occlusions and endothelial hyperactivity results Kehinde Olufemi-Aworinde, et./al Markers of Disease Severity Amongst Homozygous Sickle Cell Anaemia Attending Outpatient At Bowen University Teaching Hospital Ogbomoso, Nigeria 4747 International Journal of Medical Science and Clinical Invention, Vol. 7, Issue 02, February, 2020 from this rigid sickle shape and predisposes the red cell to destruction by macrophages and monocytes in the reticuloendothelial system, culminating in a hemolytic anaemia and vasculopathy.(5) Factors interfering with S polymerization prevents initiation of this cascade. A handful of cellular and molecular factors reduce the polymerization of the Hemoglobin S. One of the most effective is the presence of other hemoglobin within the cell. Hemoglobin F and A2 have been discovered to limit the Hemoglobin S polymerization to the greatest extent. From a clinical viewpoint, the effect of hemoglobin F on the clinical severity of sickle cell anaemia has been investigated and conclusions have been drawn. Higher hemoglobin F levels is strongly associated with fewer leg ulcers, fewer episodes of vaso-occlusive crises and longevity. Certain conditions such as compound heterozygotes for hemoglobin S and hereditary persistence of hemoglobin F have hemoglobin F concentrations of about 20% in each sickle erythrocyte. These populations are asymptomatic with only minor laboratory findings. (6) Hemoglobin A2 is another hemoglobin that inhibits polymerization of S; elevated levels are seen in Sβ thalassemia. Sβ thalassemia have fewer dense and irreversibly sickled, increased erythrocyte lifespan compared with homozygous SS (7). Concerning cellular modifiers, leukocytosis is a risk factor for early death in adults and children. Leukocytosis, in the absence of infection, is common in Sickle cell anaemia and predicts the likelihood of stroke, acute chest syndrome, and an overall increase in mortality. Neutrophils and monocytes are activated in sickle cell anaemia. These activated leukocytes promote vascular inflammation and vessel damage. When these activated leukocytes are reduced, the frequency of sickling is reduced. (8) dehydration of red cells encourages polymerization of Hemoglobin S. A higher MCHC indicative of cellular dehydration has been positively correlated with the percentage of dense irreversibly sickled cells. We aimed to record the status of the pediatric sickle cell anaemia population in our environment and correlate clinical and laboratory features with disease severity. Materials and Methods The Bowen university Teaching Hospital Human Research Ethical Review Board approved this study. Informed consent was obtained from each parent or guardian of the subjects in addition assent was obtained from subjects older than 10 years of age. The patients were recruited using the simple random technique of sampling. Patients included in the study were those in steady state, gave consent and was diagnosed as homozygous SCD (Haemoglobin SS) which was confirmed by alkaline haemoglobin electrophoresis. An interviewer-administered questionnaire was used to obtain biographic and clinical information from the patients, some clinical information was extracted from the clinical records of the patient. The subjects were between the ages of 3-17years. Twenty apparently healthy children between the ages of 5-17years and who were Hemoglobin AA served as controls. Informed consent was obtained from the parents and for children older than 10years, assent was obtained in addition. Five millilitres of venous blood were collected from the ante-cubital vein after cleaning with methylated spirit. The samples were separated into two different sample bottles. Both bottles one and two contained pottasium ethylene diaminetetra acetate (K EDTA) . Three millilitres of blood was put in sample bottle 1 and two millilitres was put in sample bottle 2 . Full blood count was run with bottle 1 within 30 minutes of sample collection using the Cell-Dyne 1200 haematology analyser. After which, Reticulocyte count was run with the blood from sample bottle 1. Sample bottle 2 was used to run Haemoglobin F using the highperformance liquid chromatographic method using Dionex UltiMate® 300 Rapid Separation LC System. Statistical analyses were conducted using the IBM Statistical Package for Social Sciences (SPSS) Tm version 23.0 for windows. Means of normally distributed data were obtained. student T test was used to investigate the difference between means. Pearson’s Correlates were used to investigate relationships between continuous variables. P values <0·05 were considered significant. Results A total of 40 patients were recruited into the study. Patient age ranged from 3 to 17 years (median 8.2 years) at the time of recruitment. Forty percent of the patients (40%) were males. The clinical characteristics of the patients with sickle cell anaemia are shown on table 1. Ten percent of the Kehinde Olufemi-Aworinde, et./al Markers of Disease Severity Amongst Homozygous Sickle Cell Anaemia Attending Outpatient At Bowen University Teaching Hospital Ogbomoso, Nigeria 4748 International Journal of Medical Science and Clinical Invention, Vol. 7, Issue 02, February, 2020 patients presented with a diagnosed infection in the previous year, while only five percent were hospitalized within the past year. Table 2 compared the blood indices for sickle cell anaemia with controls. The total white cell count and platelet count are significantly higher in the sickle cell group while the haematocrit and mean cell volume is significantly lower in the sickle cell group. The platelet count of the sickle cell anaemia patients was more than twice the number of the controls (444.6 X 10 9 /L vs 208 X 10 9 /L). The Reticulocyte count was approximately six times higher in the study group compared with the control. The mean hemoglobin F level was 7.1± 3.5 % .( Hb F for Hb A 0.5-1.5%) The hemoglobin F was negatively correlated with the platelet count but positively correlated with the total white cell count and haematocrit as noted on Table 3 Table 1: The clinical characteristics of sickle cell anaemia patients. Clinical characteris tics Response Number Percent age% Fever in the last year Yes 4 10

Markers of Disease Severity Amongst Homozygous Sickle Cell Anaemia Attending Outpatient At Bowen University Teaching Hospital Ogbomoso, Nigeria.