Uma revisão sobre o gene CD36 e sua influência em crises vaso-oclusivas de pacientes com anemia falciforme

Abstract

Sickle cell anemia is one of the most common monogenic disorders worldwide, responsible for diminishing the quality and life expectations of its carriers. The polymerization of hemoglobin S is essential for the pathophysiology of the disease, because it leads to deformity and rigidity of erythrocytes, turning them sickle-shaped, causing vaso-occlusive events and hemolytic anemia. Acute pain crisis is the clinical manifestation that most leads patients to seek medical attention and it’s a result of vaso-occlusive crises, which begins when there’s obstruction of the blood flow by sickle red blood cells. Sickle red blood cells also interact with the vascular endothelium through adhesion molecules such as CD36. Details about CD36’s expression and the impact it has on the pathogenesis of the disease are still scarce, however, review of available data in the literature made possible to corroborate the existence of an association between higher levels of CD36’s expression and blood flow impairment, through adhesive interactions between sickle red blood cells and the vascular endothelium. Such interactions are partially inhibited by the use of the drug hydroxyurea, and the injection of anti-CD36 antibodies in an animal model pointed to a reduction in the adhesiveness of sickle erythrocytes, indicating a potential therapeutic target. There are few studies that examined the possibility of genetic alterations in CD36 having an influence over its role on the pathophysiological mechanisms of sickle cell anemia, demonstrating the need for more research on this topic. Despite favoring the occurrence of vaso-oclusive crisis, it hasn’t been yet established a relation between the expression of CD36 and the clinical severity of sickle cell anemia.