Scenario of haemoglobin variants in Central-East coast of India

Haemoglobinopathies are the most common monogenic inherited disorders of erythrocytes. Carriers of haemoglobinopathies are partially protected against morbidity and mortality of falciparum malaria, resulting in their higher prevalence in tropical countries. Estimates in India show 3–17% prevalence of β-thalassaemia, but its magnitude in the Central-East coast of India, especially in Orissa is not known. One thousand fifteen cohort cases of anaemia were analysed, referred from different peripheral hospitals and medical colleges and hospitals in the Central-East coast of India during 1994 to 2003. Background data of each individual were recorded like age, sex, caste, place of origin, consanguinity, etc. Standardized laboratory procedures and techniques were followed. Most common haemoglobinopathies observed out of 1015 cases were: sickle cell trait (29.8%), sickle cell disease (7.5%), sickle cell-β-thalassaemia (1.7%), β-thalassaemia trait (18.2%), β-thalassaemia major (5.3%), thalassaemia intermedia (0.9%), Hb E trait (0.9%), Hb E disease (0.3%), Hb Eβ-thalassaemia (0.7%), Hb D trait (0.2%) and SD disease (0.2%). Sickle cell disorders with high level of foetal Hb were common in general castes (0.3–20.7%), scheduled castes (0–8.9%) and scheduled tribals (0– 5.5%). Transfusion-dependent β-thalassaemia syndrome was prevalent among Brahmins, Karans, Khandayats, Telis, etc. Most of the cases belong to Anugul district, followed by Khurda, Nayagarh, Kandhamal, Cuttack, Jajpur, Dhenkanal, Ganjam, Keonjhar, Mayurbhanj, etc. The heterogeneous population harbours almost all major haemoglobinopathies in general castes, scheduled castes and tribes, belonging to coastal and southwestern regions of Orissa. This study provides a comprehensive database on the pattern of spectrum of haemoglobinopathies in the Central-East coast of India.