A study of Blood indices in Sickle Cell Disorder

Abstract

BACKGROUND: Sickle cell disorder is second most common hemoglobinopathy after Thalassemia. Patients present with wide spectrum of disorders because of a single-point mutation in which thymine substitute for adenine, thereby encoding valine-instead of glutamine in sixth position of beta chain. Haemoglobin S causes polymerization of haemoglobin and red cell sickles on exposure to low oxygen tension and not sickle on oxygenation. Hemolysis occurs due to quantitative and qualitative changes in red blood cell. Hemolysis could be intravascular as well as extravascular which affect the Indices of Red blood cells resulting in low Hb, low RBC count and variations in RBC indices. AIMS and OBJECTIVE: The study was conducted to examine the hematological profile in patients of sickle cell disorder. To study Mentzer Index and Srivastva Index in patients with sickle cell disorderand to assess whether there is prevalence of iron deficiency anaemia with sickle cell disorders. METHODOLOGY: This was a prospective observational study in which patients of sickle cell disorder confirmed by HPLC (High performance liquid chromatography) were taken and those with history of blood transfusion in past 3 months or with HIV infection was excluded. Total 537 patients were included in this study. RESULT: The mean Hb of the study population was 9.87±2.14%.The mean MCV was 73±9fl. The mean MCH was 31.68±12.87%.The mean PCV was 32±8.15%.The mean MCHC was 31.68±12.8%.The mean Srivastava Index was 5.81±2.83. The mean Mentzer Index was 18.47±8.81.