Fetal Hemoglobin Levels in Patients With Sickle Cell Disorders and its Correlation With Disease Severity

Rashmi N. Nagdeve; Prashant L. Patil

Volume : V, Issue : I, January - 2015

Fetal Hemoglobin Levels in Patients With Sickle Cell Disorders and its Correlation With Disease Severity

Rashmi N. Nagdeve, Prashant L. Patil

Abstract :

Background: Fetal haemoglobin (HbF) levels show a wide variation in sickle cell patients throughout world resulting in variable disease severity. Though the condition is prevalent in India, there are only few studies that deal with HbF levels in these patients Aim: To study HbF levels in patients with sickle cell disorders (sickle cell trait and sickle cell disease) and its correlation with disease severity. Methods: Patients above 12–years of age with sickle cell disorders were included in the present study. A detailed demographic and clinical history was recorded. Quantitative estimation of HbF was done in each patient by alkali denaturation test. HbF levels were then correlated with vaso–occlusive episodes. Results: 100 patients had sickle cell disease and 138 had sickle cell trait. The mean age of presentation was higher in AS patients (24.89± 7.1 years) as compared to SS patients (22.22±7.9 years). Total 87.39% patients presented with joint pains while 11.34% patients were asymptomatic. Complications of sickle cell disease were noted in 2.94% patients. Mean HbF levels were 1.88±3.27% in AS group and 9.22±6.12% in SS group. In AS group, when HbF levels were studied in relation to number of VOC per year i.e. up to 3 per year and >3 per year, the difference is found to be significant. Similar correlation in SS group found to be significant. In this group, a strong negative correlation was found between HbF levels and VOC on application of correlation coefficient. Conclusions: The mean HbF levels were high in both AS and SS patients than in normal patients Also, there was a strong negative correlation between number of vaso–occlusive crises and HbF levels.