Molecular and Pathophysical studies of Vaso Occlusive crisis in sickle cell disordered patients of Odisha

Abstract

Sickle cell disorder (SCD) is a monogenic recessive â-globin gene disorder, affecting 30 million people worldwide. It is a major public health problem, associated with significant morbidity and mortality. This disorder is highly prevalent in many states of India with frequency varies from 5-40%, particularly along the central latitudinal belt. It is also highly prevalent in the western parts of Odisha State. Although a single gene disorder, the clinical effects of sickle cell disease make it behaves like a multigenic disorder. The pathological features vary from asymptomatic states to severe symptoms and lead to even death of patients. Among the pleiotropic effect of SCD, Vasso-occlusive Crisis (VOC) is the most common presenting symptom. The sickle RBCs (SRBCs) are the fundamental cause of vascular occlusions which is caused due to the polymerization of the globin protein inside the RBCs. The process of polymerization or sickling of the RBCs is a reversible reaction following the reoxygenation of the hemoglobin. Repeated cycles of sickling and unsickling processes by the formation and degradation of HbS polymer, damages the red cell membrane of the SRBCs. HbS polymerization is associated with cellular dehydration (reduction of ion and water content), increased viscosity (red cell density), and transient increase in intracellular calcium. Consequently, SRBCs become adhesive and inflexible (rigid) causing premature destruction. The decreased life span of SRBCs creates the condition of chronic hemolytic anemia and the blockage at the capillaries leads to tissue hypoxia and subsequent organ damage.