Coagulation abnormalities of sickle cell disease: Relationship with clinical outcomes and the effect of disease modifying therapies
D Noubouossie, NS Key, KI Ataga - Blood reviews, 2016 - Elsevier
Sickle cell disease (SCD) is a hypercoagulable state. Patients exhibit increased platelet
activation, high plasma levels of markers of thrombin generation, depletion of natural
anticoagulant proteins, abnormal activation of the fibrinolytic system, and increased tissue
factor expression, even in the non-crisis “steady state.” Furthermore, SCD is characterized
by an increased risk of thrombotic complications. The pathogenesis of coagulation activation
in SCD appears to be multi-factorial, with contributions from ischemia–reperfusion injury and …
activation, high plasma levels of markers of thrombin generation, depletion of natural
anticoagulant proteins, abnormal activation of the fibrinolytic system, and increased tissue
factor expression, even in the non-crisis “steady state.” Furthermore, SCD is characterized
by an increased risk of thrombotic complications. The pathogenesis of coagulation activation
in SCD appears to be multi-factorial, with contributions from ischemia–reperfusion injury and …
