Table 1

Characteristics of the pathogenic sickle red cell

Red cell characteristicEffects
Cell dehydration Increased dynamic rigidity; increased hemoglobin polymer formation and sickling; increased blood viscosity 
Hemoglobin polymer formation Sickle shape; mechanical obstruction of small-caliber vessels; hemolysis; vaso-occlusion 
Young age Increased expression of adhesion receptors; increased content of signaling molecules; activation of adhesion receptors 
Surface phosphatidylserine exposure Thrombogenic potential; activation of coagulation cascade; adhesion 
Adhesive properties Abnormal interactions with other blood cells (monocytes, neutrophils, platelets) and endothelium; vaso-occlusion; inflammation 
Oxidatively damaged membrane Defect in NO transport and delivery; abnormal cell rheology; vasoconstriction; inflammation 
Abnormal cell-cell signaling Activation of endothelial cells and monocytes; inflammation; vasoconstriction 
Red cell characteristicEffects
Cell dehydration Increased dynamic rigidity; increased hemoglobin polymer formation and sickling; increased blood viscosity 
Hemoglobin polymer formation Sickle shape; mechanical obstruction of small-caliber vessels; hemolysis; vaso-occlusion 
Young age Increased expression of adhesion receptors; increased content of signaling molecules; activation of adhesion receptors 
Surface phosphatidylserine exposure Thrombogenic potential; activation of coagulation cascade; adhesion 
Adhesive properties Abnormal interactions with other blood cells (monocytes, neutrophils, platelets) and endothelium; vaso-occlusion; inflammation 
Oxidatively damaged membrane Defect in NO transport and delivery; abnormal cell rheology; vasoconstriction; inflammation 
Abnormal cell-cell signaling Activation of endothelial cells and monocytes; inflammation; vasoconstriction 

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