Study of sickle cell disease by hematological parameter and HPLC at tertiary care center

Abstract

Background: Sickle Cell Disease is an inherited disease characterized by the presence of an abnormal hemoglobin called hemoglobin S (HbS) which is mutated form of hemoglobin. In this study we want to profile various types of hemoglobin’s and their relative percentage in sickle cell cases. Also, we will analyse RBC indices such as Hb, MCV, MCH, MCHC. Ams and Objectives: To study various hematological parameters and peripheral blood smear findings of sickle cell disease, to determine incidence of sickle cell disease according to age and sex group and classify sickle cell disease based on HPLC findings.Methods: We analyzed blood from 145 patients suspected to have Sickle cell hemoglobinopathies and subjected it to Sickling screening test. All positive cases will be subjected to HPLC to separate hemoglobin and CBC analysis was done to check RBC indices. Results: 51% patients were having sickle cell trait (AS), 9% showed sickle cell disease (SS) and 40% were sickle beta thalassemia (Sβ). In sickle cell disease patients, there were significantly higher levels of HbA2, HbF and HbS and significantly lower levels of HbA. Both sickle cell trait and sickle cell disease patients had significantly lower levels of MCH. Conclusion: Peripheral blood smear examination, Sickling test and HPLC findings are very important investigations to diagnose Sickle Cell Disease. Sickle cell disease is very common in our populations mainly in rural area. High index of suspicion should be maintained when these parameters are on lower side, especially in population who is prone to have sickle cell disorders.