Sickle Hemoglobinopathy in West Bengal, India: Review of Literature and Future Direction

Abstract

Sickle cell disease (SCD) was the first example of a ‘molecular disease’. SCD patients experience considerable morbidity from both acute and chronic sequelae; most severe cases can be fatal within the first few years of life without effective treatment or with sub-optimal treatment. In India, reported sickle-cell allele frequency is 10% spanned central India. West Bengal is the first state in India which had launched the State Thalassaemia Control Programme (STCP) for prevention of thalassemia since 2008. The present systematic review was made to know the community load of sickle hemoglobinopathy in West Bengal, an eastern state in India. Among all, as per the recently published, largest study screened for hemoglobinopathies over a period of 10 years, the estimated community load of sickle hemoglobinopathy in West Bengal would be sickle cell trait/carrier (703,010 cases; 0.77%), HbS beta thalassemia (45,650 cases; 0.05%) and SCD (36,520 cases; 0.04%). The National Health Mission, Government of India in 2016 have published the guidelines for ‘prevention and control of hemoglobinopathies in India’. Data from many functioning thalassemia control units (TCUs) in West Bengal are not yet published; therefore not available for the current review. All the TCUs in West Bengal should publish their data to help the Government in planning comprehensive management of SCD including other thalassemia cases.