The compatibility measurement of Mentzer, England & Fraser, Shine & Lal, and Srivastava indices to the hemoglobin electrophoresis result for beta thalassemia trait screening

Abstract

Background: Thalassemia is an autosomal recessive disease. It occurs due to abnormalities in hemoglobin synthesis, causes the imbalance of globin production. Identification of thalassemia’s carrier plays a role to prevent disease’s inheritance. Diagnostic approaches regarding anemia, especially in families are important because they are prone to inherit the disease. This study was aimed to know the compatibility of Mentzer, England & Fraser, Shine & Lal, and Srivastava indices with hemoglobin electrophoresis in transfusion-dependent thalassemia patient’s family. Methods: This was a cross sectional study by obtaining routine hematology and hemoglobin electrophoresis from thalassemia major patient’s family screening. The data then analyzed with Kappa and chi-square to get the compatibility and significance between variables. Data were analyzed using Microsoft Excel and SPSS version 17 for Windows Results: From 99 subjects, most of them are women aged 18-55 years. Beta thalassemia trait based on Mentzer, England & Fraser, Srivastava, and Shine & Lal was in 46,5%, 55,6%, 55,6%, and 54,5% research subjects respectively. Hemoglobin electrophoresis results showed that beta thalassemia trait was in 47.5% subjects. Kappa test showed good and significant compatibility result of hemoglobin electrophoresis with Mentzer (k=0,663,p= 0,0001) and England & Fraser (k=0,636,p=0,002) indices, also moderate and significant compatibility for Srivastava (k=0,558,p=0,018) and Shine & Lal indices(r=0,527,p= 0,015) with hemoglobin electrophoresis Conclusions: There were compatible and significant results between Mentzer and England & Fraser with hemoglobin electrophoresis. Meanwhile, Srivastava and Shine & Lal indices have medium compatibility so that they can be used as an initial screening for beta thalassemia trait in transfusion-dependent thalassemia patient’s family