Helen Fogarty, Azaz Ahmad, Ferdows Atiq, Dearbhla Doherty, Soracha Ward, Ellie Karampini, Aisling M Rehill, Gemma Leon, Ciara Byrne, Rosena Geoghegan, Helena Conroy, Mary B Byrne, Ulrich Budde, Sonja Schneppenheim, Ciara Sheehan, Noel Ngwenya, Ross Ian Baker, Roger J S Preston, Emma Tuohy, Corrina B McMahon, James S O'Donnell
Previous studies have reported elevated von Willebrand factor (VWF) levels in patients with sickle cell disease (SCD) and demonstrated a key role for the VWF-ADAMTS13 axis in the pathobiology of SCD vaso-occlusion. Although blood transfusion is the gold standard for stroke prevention in SCD, the biological mechanisms underpinning its improved efficacy compared to hydroxycarbamide are not fully understood. We hypothesized that the improved clinical efficacy of blood transfusion might relate to differences in endothelial cell activation and VWF-ADAMTS13 axis dysfunction...
September 29, 2023: Blood Advances