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Review
. 2016 Jul;30(4):245-56.
doi: 10.1016/j.blre.2015.12.003. Epub 2015 Dec 24.

Coagulation abnormalities of sickle cell disease: Relationship with clinical outcomes and the effect of disease modifying therapies

Denis Noubouossie  1 , Nigel S Key  1 , Kenneth I Ataga  2
Affiliations
Review

Coagulation abnormalities of sickle cell disease: Relationship with clinical outcomes and the effect of disease modifying therapies

Denis Noubouossie et al. Blood Rev. 2016 Jul.

Abstract

Sickle cell disease (SCD) is a hypercoagulable state. Patients exhibit increased platelet activation, high plasma levels of markers of thrombin generation, depletion of natural anticoagulant proteins, abnormal activation of the fibrinolytic system, and increased tissue factor expression, even in the non-crisis "steady state." Furthermore, SCD is characterized by an increased risk of thrombotic complications. The pathogenesis of coagulation activation in SCD appears to be multi-factorial, with contributions from ischemia-reperfusion injury and inflammation, hemolysis and nitric oxide deficiency, and increased sickle RBC phosphatidylserine expression. Recent studies in animal models suggest that activation of coagulation may contribute to the pathogenesis of SCD, but the data on the contribution of coagulation and platelet activation to SCD-related complications in humans are limited. Clinical trials of new generations of anticoagulants and antiplatelet agents, using a variety of clinical endpoints are warranted.

Keywords: Coagulation activation; Complications; Hemolysis; Inflammation; Platelet activation; Sickle cell disease.

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Conflict of interest statement

Conflict of interest

The authors declare no conflict of interest associated to this article.

Figures

Figure 1
Figure 1. Pathogenesis of thrombosis in sickle cell disease
RBC - Red blood cells; isRBC - Irreversibly sickled red blood cells; PLT - Platelets; MP - Microparticles; cfDNA - Cell-free DNA; NETs - Neutrophil extracellular traps; NO - Nitric oxide; IRI - Ischemia reperfusion injury; TF - Tissue factor; PS - phosphatidylserine; EC - Endothelial cell; vWF - von Willebrand factor; FVIII - Factor VIII; FXa - Activated factor X.
See this image and copyright information in PMC

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