Table 2

Consequences of intravascular hemolysis in the mechanisms of thrombosis in PNH

1. Release of free hemoglobin resulting in: 
 a. Platelet activation 
 b. Thrombophlebitis 
 c. NO depletion with consequences of: 
  i. Vasoconstriction 
  ii. Platelet activation and aggregation 
  iii. Increased expression of cellular adhesion molecules 
  iv. Secretion of procoagulant proteins 
  v. Ischemia-reperfusion injury 
  vi. Endothelial proliferation 
  vii. Increased inflammatory markers 
  viii. Factor XIII activation 
  ix. Increased levels of thrombin-antithrombin complexes and fibrin split products 
 d. Inhibition of ADAMTS13 
 e. Direct endothelial dysfunction 
 f. Activation of endothelial cells and release of microparticles 
 g. Increased levels of tissue factor 
 h. Increased levels of reactive oxygen species 
2. Release of arginase resulting in NO depletion 
3. Release of procoagulant red cell microparticles 
1. Release of free hemoglobin resulting in: 
 a. Platelet activation 
 b. Thrombophlebitis 
 c. NO depletion with consequences of: 
  i. Vasoconstriction 
  ii. Platelet activation and aggregation 
  iii. Increased expression of cellular adhesion molecules 
  iv. Secretion of procoagulant proteins 
  v. Ischemia-reperfusion injury 
  vi. Endothelial proliferation 
  vii. Increased inflammatory markers 
  viii. Factor XIII activation 
  ix. Increased levels of thrombin-antithrombin complexes and fibrin split products 
 d. Inhibition of ADAMTS13 
 e. Direct endothelial dysfunction 
 f. Activation of endothelial cells and release of microparticles 
 g. Increased levels of tissue factor 
 h. Increased levels of reactive oxygen species 
2. Release of arginase resulting in NO depletion 
3. Release of procoagulant red cell microparticles 
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