Novel Sickle Cell Disease Therapies: Targeting Pathways Downstream of Sickling

Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by hemolytic anemia, frequent painful episodes, poor quality of life, end organ damage and a shortened lifespan. While the seminal event is the polymerization of the abnormal hemoglobin, the downstream pathophysiology of vaso occlusion (VOC) results from heterotypic interactions between the altered, adhesive sickle cell RBC ′s, neutrophils, endothelium and platelets. Ischemia reperfusion injury, hemolysis and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system.
Source: Seminars in Hematology - Category: Hematology Authors: Source Type: research