The hemoglobinopathies include all genetic diseases of hemoglobin (Hb) and fall into two main groups: the thalassemias and structural hemoglobin variants (abnormal hemoglobins). Thalassemia is a public health problem in Malaysia. About 4.5% of the Malays and Chinese are β-thalassemia carriers. We performed hemoglobin analysis on a total of 499 patients from a Government Hospital and Health Clinics in the state of Perlis, Malaysia. About 91.4% of the patients were Malays. All patients had microcytic hypochromic anemia except for a few who went for thalassemia screening. Female patients outnumbered male patients in the ratio of 3.5:1. About 75.7% of the female patients were of childbearing age (17 - 40 years) and a majority of them were there for their antenatal checkup. Using our screen tests (full blood count, high performance liquid chromatography (HPLC), and agarose gel electrophoresis), the common hemoglobinopathies detected were HbE trait (19.3%), β-thalassemia trait (14.6%), HbH disease (1.8%), Hb Constant Spring (1.6%), Homozygous HbE (1.4%), and HbE- β-thalassemia (1.4%). Thalassemia is preventable through screening and education programmes, and prenatal diagnosis. Thalassemia screening is provided free of charge at various government hospitals and health clinics throughout the country.
Key words: Hemoglobinopathies screening, β-thalassemia trait, HbE trait, Thalassemic diseases