Thrombospondin-1 inhibits ADAMTS13 activity in sickle cell disease

Thrombospondin-1 inhibits ADAMTS13 activity in sickle cell diseaseSickle cell disease (SCD) is characterized by increased cellular adhesiveness, hemolytic anemia, hemostatic activation, vaso-occlusion and thrombosis.We found that patients with SCD in steady state have elevated plasma levels of the platelet protein thrombospondin-1 (TSP1) and that these levels are associated with vaso-occlusive complications and a history of acute chest syndrome (ACS). 1 Critically ill patients with ACS develop thrombocytopeniaSevere vaso-occlusive episode is defined as an acute pain episode leading to an emergency department visit or hospitalization in the 12 months prior to the study lab draw. 1 Unless otherwise indicated. 2From Wilcoxon's rank sum test or Pearson's c 2 test of independence. 3Number (percentage) of patients with ul-VWF multimers. 4Patients on hydroxyurea were on a stable dose over the preceding 3 months. 5MRI-detectable cerebrovascular accidents (overt strokes and silent infarcts).