Sickle cell anemia: An update on diagnosis, management and prevention strategies

Sickle cell anemia is the most common disease entity of all the monogenic disorders. This is an autosomal recessive disorder. HbS polymerization, vaso-occlusion, and hemolytic anemia are central to the pathophysiology of sickle cell disease, they precipitate a cascade of pathologic events, which in turn lead to a wide range of complications. The disease is particularly more prevalent in certain regions of the country like Odisha, Madhya Pradesh, Tamilnadu and has a significant impact on morbidity. Community based approach by providing availability of screening tests, & pre-marital and pre-pregnancy counselling should be initiated to reduce the disease burden in the society.