ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso-occlusive crisis of sickle cell disease.

Background: Sickle cell disease (SCD) is characterized by vaso-occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD-related endothelial injury.

Objectives: To decipher the role of VWF and its specific-cleaving metalloprotease, ADAMTS13, in the vaso-occlusive and thrombotic process of SCD.