HbF in HbE/β-thalassemia: A clinical and laboratory correlation

Abstract

Introduction: Fetal hemoglobin (HbF) is the predominant hemoglobin in red cells during fetal life. Just after birth, the level of HbF decreases gradually to <1%, and is replaced mainly by adult hemoglobin (HbA) (~97%). However, higher HbF levels could be associated with HbE/β-thalassemia, a complex thalassemia intermedia with a diverse clinical severity ranging from mild-to-severe anemia. This study investigates the correlation of HbF level with the clinical and laboratory data of HbE/β-thalassemia individuals. Methods: Peripheral blood samples from 30 HbE/β-thalassemia subjects were subjected to a full blood count, genomic as well as quantitative real-time polymerase chain reaction gene expression studies. Statistical analyses were performed using SPSS 17.0. Results: HbF levels were influenced by age, mean cell volume (MCV), mean cell hemoglobin (MCH), HbA, β-globin, and α/β-globin expressions. Discussion: HbF production is affected by the α/β-globin chain imbalance due to the lack of β-globin gene expression as well as inversely correlates to the amount of functional hemoglobin available in the cells.