An evaluation of risk for severe β - thalassemia in the public health area 7 of Thailand

Abstract

          Thalassemia disease is high incidence in Thailand, in Thai people,600,000 case of disease and about

3-9%of the Thalassemia are β-thalassemia disease and 20-30% of case are α- thalassemia disease. This disease is a group of inherrited  autosomal recessive blood disorder, although persons who carry the minor form β or α-thalassemia gene and/or abnormal globin gene but they usually have no sign or no symptoms. Many Thai peoples have these genes (or call carrier) and gene-genes interactions in this population can lead to several thalassemia syndromes. The common thalassemia carriers are usually founded such as Hemoglobin E trait, β-thalassemia trait, α-thalassemia trait, α-thalassemia-2  and Hemoglobin constanspring trait. The frequency of thalassemia types were varied in different parts of Thailand. Thai national policy for prevention and control thalassemia emphasized on 3 severe thalassemia disease  such as homozygous β-thalassemia, β-thal/ Hb E  and Hemoglobin Bart’s hydrop fetalis. These serious cases have severe symptoms and early death. The effectiveness of prevention and control program in communities can reduce the new serious patients.  The study aims to screen for β-thalassemia trait by using Automatic Cation Exchange Chromatography (HPLC and LPLC). One hundred blood samples which had   Hb A₂ value was equal or more than 4.0%, were detected to the abnormal globin genes by using the Allele Specific PCR (ASPCR) and Gene Sequencing method was used as the confirmatory test. The result  found that  65.5% of all, were severe β- thalassemia trait and 100% of these results were similar between ASPCR technique and Gene Sequencing. This present study is useful and applicable for the effective prevention and control of overall thalassemia diseases.