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

ABSTRACT

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.