Table 1.

Biomarkers of pathogenic processes in SCD

TypeBiomarker
Hemolysis Routine laboratory LDH, bilirubin, reticulocyte count 
Combined Hemolytic index 
Molecular Free heme and plasma hemoglobin, arginine/arginase ratio 
Cell based Dense RBC fraction 
Endothelial dysfunction Physiological Elevated pulse pressure 
Routine laboratory VWF, flow-mediated vasodilation 
Molecular Circulating microparticles 
Cell based Circulating endothelial cells 
Sterile inflammation Routine laboratory Erythrocyte sedimentation rate and C-reactive protein 
Molecular Nucleosomes; elastase-α1-antitrypsin; chromatin-binding protein HMGB1; NLRP3; cytokines IL-6, IL-8, and IL-1β 
Hemostatic activation Routine laboratory d-dimer; ADAMTS13 activity 
Molecular Active VWF, thrombin-antithrombin complexes, thrombospondin 1 
Cell based Circulating endothelial cells with tissue factor phenotype 
Oxidant stress Molecular Oxidized glutathione, glutamine 
Blood hyperviscosity Routine laboratory High hemoglobin 
Cellular hyperadhesion Routine laboratory White blood cell count 
Molecular Soluble E-selectin, P-selectin, and MAC-1 
Cell based RBC phosphatidylserine and integrin α4βi (VLA4) 
TypeBiomarker
Hemolysis Routine laboratory LDH, bilirubin, reticulocyte count 
Combined Hemolytic index 
Molecular Free heme and plasma hemoglobin, arginine/arginase ratio 
Cell based Dense RBC fraction 
Endothelial dysfunction Physiological Elevated pulse pressure 
Routine laboratory VWF, flow-mediated vasodilation 
Molecular Circulating microparticles 
Cell based Circulating endothelial cells 
Sterile inflammation Routine laboratory Erythrocyte sedimentation rate and C-reactive protein 
Molecular Nucleosomes; elastase-α1-antitrypsin; chromatin-binding protein HMGB1; NLRP3; cytokines IL-6, IL-8, and IL-1β 
Hemostatic activation Routine laboratory d-dimer; ADAMTS13 activity 
Molecular Active VWF, thrombin-antithrombin complexes, thrombospondin 1 
Cell based Circulating endothelial cells with tissue factor phenotype 
Oxidant stress Molecular Oxidized glutathione, glutamine 
Blood hyperviscosity Routine laboratory High hemoglobin 
Cellular hyperadhesion Routine laboratory White blood cell count 
Molecular Soluble E-selectin, P-selectin, and MAC-1 
Cell based RBC phosphatidylserine and integrin α4βi (VLA4) 

ADAMTS13, ADAM metallopeptidase with thrombospondin type 1 motif 13.

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