title: Hypercoagulability in sickle cell disease: a curious paradox.
abstract: 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. Similarly, exposure of surface markers such as CD62P and CD40L, along with increased circulating levels of thrombospondin, signal platelet activation. In addition to its effects on the cleavage of fibrinogen and its ability to activate platelets, the increase in circulating thrombin levels, with its wide-ranging effects on endothelial cells and blood vessels, may be important in the pathophysiology of sickle cell disease. 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.
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, University of North Carolina Comprehensive Sickle Cell Program, Chapel Hill, North Carolina 27599-7305, USA. kataga@med.unc.edu
authors.forename: Kenneth I
authors.initials: KI
authors.lastname: Ataga
authors.forename: Eugene P
authors.initials: EP
authors.lastname: Orringer
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood* / Q000473:pathology / Q000503:physiopathology*; D000925:Anticoagulants / Q000627:therapeutic use; D001777:Blood Coagulation / Q000187:drug effects / Q000502:physiology; D004730:Endothelium, Vascular / Q000187:drug effects / Q000378:metabolism / Q000503:physiopathology; D006801:Humans; D009336:Necrosis; D015539:Platelet Activation / Q000187:drug effects / Q000502:physiology; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D019851:Thrombophilia / Q000097:blood* / Q000473:pathology / Q000503:physiopathology*; D013925:Thromboplastin / Q000187:drug effects / Q000502:physiology; D013927:Thrombosis / Q000097:blood / Q000188:drug therapy / Q000503:physiopathology
chemical_list: D000925:Anticoagulants; D010975:Platelet Aggregation Inhibitors; D013925:Thromboplastin