Clinical Presentation and Electrophoretic Patterns of Hereditary Haemoglobin Disorders in Adults, a Study at Dinajpur Medical College Hospital

Genetic defects of haemoglobin are the most common genetic disorders and affect around 7% of world’s population. Thalassaemias and haemoglobinopathies have been sporadically found in every ethnic group and geographic region. They occur with particularly high frequency from the shores of the Mediterranean and Africa through the Middle East, Bangladesh, India, Sri Lanka, Myanmar, Thailand and other countries of Southeast Asia. The study was designed to find out the clinical presentation and electrophoretic patterns of hereditary haemoglobin disorders among adult patients admitted at Dinajpur Medical College Hospital. A total number of 60 adult patients of either sex irrespective of age having evidence of haemolysis in the peripheral blood were included in this observational study from July 2011 to July 2012. Patients were diagnosed by complete blood count, morphological blood film examination and haemoglobin electrophoresis on cellulose acetate at pH 8.6. Detailed history was taken along with thorough physical examination. Other relevant investigations like serum bilirubin, ALT, HBsAg, Anti-HCV, Ultrasonography of whole abdomen were done when indicated. Among 60 patients, Hb E trait was (41.67%), HbE disease (30%), HbE-beta thalassaemia was (23.33%), beta thalassaemia minor (3.33%) and Hb H disease (1.67%). It is evident that, hereditary haemoglobin disorders are quite common in Bangladesh and these disorders are inherited in an autosomal recessive Mendelian pattern affecting both males and females. Since these disorders are incurable, emphasis must shift from treatment of the affected child to prevention of such births in future.