Table 1.

Hemostatic parameters in thalassemia and SCD

Assayβ-TMβ-TIα-ThalassemiaSCD
Platelet Life span Short Short   
 Aggregation Impaired Impaired  Impaired 
 Urinary TXA2 High High  High 
 Circulating platelet aggregates Present Present Present Present  
 CD62, CD63 High High  High 
 PF3 High High High  
 PF4, β-TG High High  High  
Vascular endothelium Thrombomodulin High High  High 
 ICAM-1 High High  High 
 VCAM-1 High High  High 
 VWF High High  High 
 E-selectin High High  High  
 Urinary PGI2 High High  High  
RBC Annexin V binding Increased Increased  Increased  
 Thrombin generation Increased Increased  Increased  
Coagulation factors Factor II Low Low   
 Factors V, VII, X Normal Normal   
Coagulation inhibitors Protein C (antigen, activity) Low Low  Low  
 Protein S (free) Low Low  Low 
 ATIII Normal Low  Normal 
 HCII Low    
Thrombin generation TAT High High High High 
 F1,2 Normal High  Normal 
 FPA  High  High 
 D-dimer  High  High 
Assayβ-TMβ-TIα-ThalassemiaSCD
Platelet Life span Short Short   
 Aggregation Impaired Impaired  Impaired 
 Urinary TXA2 High High  High 
 Circulating platelet aggregates Present Present Present Present  
 CD62, CD63 High High  High 
 PF3 High High High  
 PF4, β-TG High High  High  
Vascular endothelium Thrombomodulin High High  High 
 ICAM-1 High High  High 
 VCAM-1 High High  High 
 VWF High High  High 
 E-selectin High High  High  
 Urinary PGI2 High High  High  
RBC Annexin V binding Increased Increased  Increased  
 Thrombin generation Increased Increased  Increased  
Coagulation factors Factor II Low Low   
 Factors V, VII, X Normal Normal   
Coagulation inhibitors Protein C (antigen, activity) Low Low  Low  
 Protein S (free) Low Low  Low 
 ATIII Normal Low  Normal 
 HCII Low    
Thrombin generation TAT High High High High 
 F1,2 Normal High  Normal 
 FPA  High  High 
 D-dimer  High  High 

The various assays are detailed in the text.

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