The American journal of medicine
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There is evidence of activation of both blood coagulation and platelets in sickle cell disease. For example, plasma samples obtained in the steady state and during painful crisis demonstrate high levels of thrombin generation, depletion of anticoagulant proteins, and abnormal activation of the fibrinolytic system. ⋯ Therefore, treatments that could decrease thrombin generation or platelet activation may be beneficial in both the treatment of sickle cell disease and the prevention of complications that characterize this genetic disorder. This review discusses hypercoagulability in the various forms of sickle cell disease, including homozygous sickle cell anemia, hemoglobin SC disease, hemoglobin SD disease, and sickle cell-beta-thalassemia.