Clot Club: Hypercoagulation During Sickle Cell Disease

Posted: May 25, 2021

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Clot Club: Hypercoagulation During Sickle Cell Disease

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Sickle cell disease (SCD) is an inherited hemoglobin disease that disrupts the ability of red blood cells (RBCs) to deliver oxygen throughout the body. RBCs are normally round and flexible, which allows them to flow easily through the vasculature. However, in SCD, RBCs are crescent-shaped due to the expression of an atypical sickle hemoglobin variant (HbS). The abnormal shape of sickle RBCs can cause them to clump together and block blood flow and promote thrombosis. Due to the hypercoagulable state of SCD, thrombotic complications can drive ischemic stroke, venous thromboembolism, severe pain episodes, and organ damage.

Coagulation factors are key initiators in the pathogenesis of SCD. Interestingly, the coagulation system can be activated and elevated in individuals with SCD, even in the absence of vascular occlusions.

Coagulation Markers in Research

It is suggested that aberrant coagulation and platelet overactivation contribute to the pathogenesis of SCD. Further research and clinical trials are needed to explore the mechanisms driving abnormal coagulation in SCD and to identify effective therapies to treat it.

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Coagulation Factors Components Function Biomarkers and Methods of Evaluation Activity in Sickle Cell Disease
Extrinsic Pathway Tissue factor (TF) FVII Primary pathway to initiate coagulation following vascular endothelial injury Prothrombin time (PT) Increased TF expression
Intrinsic Pathway Prekallikrein HMWK,
Activated by exposed collagen in the subendothelium and functions to initiate clot formation Activated partial thromboplastin time (aPTT) Decreased Prekallikrein, FXII, and HMWK plasma levels
Increased levels of procoagulant microparticles
Common Pathway FV, FVIII, FIX, FX
Catalysts in the final steps of coagulation to cleave fibrinogen and form blood clots Thrombin Generation Assays (TGA), TAT, PAP, F1.2, D-dimers, Fibrinogen Increased thrombin levels
Fibrinolysis Plasmin Enzymatic degradation of fibrin in blood clots uPA, tPA, PAI-1, D-dimers Increased fibrinolytic activity
Platelet Activation Collagen Fibrinogen,
Platelets activate and aggregate to form a platelet plug vWF, ADAMTS-13 Increased platelet numbers, platelet activation, and platelet aggregation
Complement System C3a
Activated by the coagulation system and contributes to thrombus formation C3a and C5a Increased C3a and C5a expression
Anticoagulant Proteins Protein C
Protein S
Negative feedback to regulate coagulation and inflammation Protein C
Protein S
Decreased Protein C and Protein S levels


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Instrument Fully Automated Coagulation Analyzer Clot Growth & Fibrinolysis Dynamics Primary Hemostasis and Platelet Function Testing
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Ceveron® series, Thrombodynamics®, and Atlas® PST are for research use only. Not for use in diagnostic procedures.

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