Interplay between coagulation and vascular inflammation in sickle cell disease

Br J Haematol. 2013 Jul;162(1):3-14. doi: 10.1111/bjh.12336. Epub 2013 Apr 18.


Sickle cell disease is the most common inherited haematological disorder that leads to the irreversible damage of multiple organs. Although sickling of red blood cells and vaso-occlusion are central to the pathophysiology of sickle cell disease, the importance of haemolytic anaemia and vasculopathy has been recently recognized. A hypercoagulable state is another prominent feature of sickle cell disease and is mediated by activation of both intrinsic and extrinsic coagulation pathways. Growing evidence demonstrates that coagulation may not only contribute to the thrombotic complications, but also to vascular inflammation associated with this disease. This article summarizes the role of vascular inflammation and coagulation activation, discusses potential mechanisms responsible for activation of coagulation and reviews recent data demonstrating the crosstalk between coagulation and vascular inflammation in sickle cell disease.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell / blood
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / etiology
  • Blood Coagulation
  • Blood Coagulation Disorders / etiology*
  • Enzyme Activation
  • Humans
  • Protein Multimerization
  • Thromboplastin / metabolism
  • Thrombosis / etiology
  • Vasculitis / etiology*
  • von Willebrand Factor / chemistry


  • von Willebrand Factor
  • Thromboplastin