Table 1.
Alterations in the coagulation system in humans with SCD
| Biochemical evidence of coagulation activation | Increased levels | Decreased levels | Steady state | Acute crisis | Reference |
|---|---|---|---|---|---|
| FXa generation | |||||
| Tissue factor pathway | + | + | + | 18, 68 | |
| Intrinsic pathway (FXII, HMWK, prekallikrein) | − | − | 31, 32 | ||
| Thrombin/fibrin generation | |||||
| D-dimer | + | + | + | 69 | |
| Prothrombin fragment F1.2 | + | + | + | 70 | |
| Fibrinopeptide A | + | 71 | |||
| Thrombin antithrombin complexes | + | + | + | 68 | |
| Changes in anticoagulant protein levels | |||||
| Protein C and S | − | − | − | 72, 73 | |
| ATIII and heparin cofactor II | − | − | 74, 75 | ||
| Changes in fibrinolytic protein levels | |||||
| Plasminogen activator inhibitor 1 | + | + | + | 76, 77 | |
| Other plasmatic factors | |||||
| FVIII | + | + | 78 | ||
| VWF | + | + | 79 | ||
| Cellular factors contributing to coagulation | |||||
| Platelet number and size | + | + | * | 22 | |
| Platelet activation and function | + | + | + | 80 | |
| Red blood cell activation (PS exposure and adhesion) | + | + | 81 | ||
| Leukocyte activation (TF exposure) | + | + | + | 20 | |
| Endothelial activation (TF exposure and adhesion) | + | + | + | 17 | |
| Cell-derived microparticles | + | + | + | 21 |
+, increased compared with controls; −, decreased compared with controls; ATIII, antithrombin III; FVIII, factor VIII; FXa, factor Xa; FXII, factor XII; HMWK, high-molecular-weight kininogen; PS, phosphatidylserine; TF, tissue factor; VWF, von Willebrand factor.
*
Variable findings.