Background: Inherited hemoglobin disorders constitutes major bulk of genetic diseases in India. Inherited hemoglobin disorders include structural hemoglobin variants and thalassemia. Structural hemoglobin variants mostly result from amino acid substitution in either α or β chains.The cumulative gene frequency of hemoglobinopathies in India is 4.2%. Methods: A total of 150 patients were examined. We have examined all patients with anemia or suspected beta thalassemia. The patient groups were evaluated according to the clinical presentation. Results: Sickle cell trait was the most common hemoglobinopathy detected. Occurrence of hemoglobinopathies was highest in Buddhas. Pallor was the most common symptom. Conclusions: Sickle cell trait was the most common hemoglobinopathy detected. Occurrence of hemoglobinopathies was highest in Buddhas, followed by Muslim and Banjaras. Most common affected ethnic group was Buddha followed by Banjaras in sickle cell syndromes. Pallor was the most common symptom in present study [60 %], followed by jaundice [14.70 %]. Vaso-occlusive crisis [abdominal pain, joint pain] was more common in sickle /beta thalassemia than sickle cell anaemia, while jaundice was more common in sickle cell anaemia than sickle /beta thalassemia. Jaundice was found to be less common in beta-thalassemia major. Splenomegaly was more common in beta thalassemia major than sickle cell anemia. Hplc was found to be less labour intensive, rapid and more reliable for quantification of hemoglobin variants. Family studies are required to confirm the diagnosis.
