Giulia Reggiani, Maria Paola Boaro, Federica Menzato, Emiliano De Bon, Antonella Bertomoro, Alessandra Casonato, Fabrizio Lucente, Cecilia Fortino, MariaTeresa Sartori, Agostino Steffan, Maddalena Martella, Mirco D'Agnolo, Laura Sainati, Raffaella Colombatti
Adults with sickle cell trait (SCT) have a procoagulant state with increased risk of thromboembolism, but limited data are available for children. We compared the coagulation profile of children with SCT, different sickle cell disease (SCD) genotypes, and healthy controls. Compared to controls and similarly to HbSC patients, 41 SCT children (mean age 6.85 years; 20 males; 88% Africans) had a characteristic procoagulant profile: higher levels of factor VIII, von Willebrand factor (VWF) Ag and CBA, D-dimer; lower levels of ADAMTS 13 activity, ADAMTS13 activity: VWFAg, plasminogen activator inhibitor, tissue plasminogen activator...
March 30, 2024: Pediatric Blood & Cancer