Sickle cell disease (SCD) is usually caused due to the alteration of the beta-globin subunit in the primary adult haemoglobin (HbA). When both the beta-globin subunits get mutated, i.e., in the heterozygous condition, the HbS polymerizes upon deoxygenation, resulting in the erythrocytes acquiring sickle or crescent shape, known as sickling. During the initial process, the acquired erythrocytes often sway between the circular and sickle shapes when oxygen pressure is lower. However, the situation becomes critical if the change becomes irreversible resulting in permanent sickling of the erythrocytes, worsening the threat of hemolysis and vascular damage. The most common form of the disease is sickle cell anemia, where the patient is susceptible to hemolysis. Every year, thousands of cases are being reported, the majority being from Sub-Saharan Africa. Despite the genetic similarity at the site of mutation, not all patients get equally affected. If the sickle cell disease is left untreated for long, it causes neurological complications, pulmonary hypertension, cardiac disease, renal complications etc. Various methods have been developed for the treatment of the disease. This review briefly discusses sickle cell disease, its pathophysiological aspects, its causes, effects and treatment.

Keywords : Sickle Cell Anemia, Pathophysiology, Hemolysis, Thalassemia, Mutation.