Background: Thalassemia is an autosomal recessive disease. It occurs due to abnormalities in hemoglobin synthesis, causes the imbalance of globin production. Identification of thalassemiaâ€™s carrier plays a role to prevent diseaseâ€™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 patientâ€™s family.
Methods: This was a cross sectional study by obtaining routine hematology and hemoglobin electrophoresis from thalassemia major patientâ€™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) Â 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 electrophoresisConclusions: 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 patientâ€™s family