Coagulation abnormalities of sickle cell disease

Relationship with clinical outcomes and the effect of disease modifying therapies

Denis Noubouossie, Nigel S. Key, Kenneth Ataga

Research output: Contribution to journalReview article

27 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)245-256
Number of pages12
JournalBlood Reviews
Volume30
Issue number4
DOIs
StatePublished - Jul 1 2016

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Sickle Cell Anemia
Platelet Activation
Anticoagulants
Therapeutics
Platelet Aggregation Inhibitors
Phosphatidylserines
Thromboplastin
Hemolysis
Thrombin
Nitric Oxide
Animal Models
Clinical Trials
Inflammation
Wounds and Injuries
Proteins

All Science Journal Classification (ASJC) codes

  • Hematology
  • Oncology

Cite this

Coagulation abnormalities of sickle cell disease : Relationship with clinical outcomes and the effect of disease modifying therapies. / Noubouossie, Denis; Key, Nigel S.; Ataga, Kenneth.

In: Blood Reviews, Vol. 30, No. 4, 01.07.2016, p. 245-256.

Research output: Contribution to journalReview article

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