Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a single base substitution in the β-globin gene. Mutated hemoglobin polymerizes under low oxygen conditions and leads to red blood cell (RBC) membrane deformity and fragility, with resulting intra and extravascular hemolysis. Patients with SCD suffer from severe chronic hemolytic anemia and acute episodes of RBC trapping and destruction in the microvasculature (vaso-occlusive crises). Hemolysis in SCD and other hematological disorders such as paroxysmal nocturnal hemoglobinuria, thalassemia and malaria, has been associated with a clinical hypercoagulable state that leads to a high prevalence of thrombotic complications responsible for high morbidity and mortality. It is therefore critical to elucidate the link between hemolysis and thrombosis, so that effective preventive and therapeutic strategies can be developed. This review summarizes the current concepts on hemolysis-mediated thrombosis.
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