Affiliation:
1. Department of Women's and Child's Health Pediatric Hematology‐Oncology Unit Azienda Ospedaliera‐Università di Padova Padua Italy
2. UOC General Medicine Cittadella's Hospital Cittadella Italy
3. Department of Medicine Internal Medicine 1 Unit Azienda Ospedaliera‐Università di Padova Padua Italy
4. Department of Diagnostic Laboratory and Cellular Therapy Centro Riferimento Oncologico Aviano Italy
Abstract
AbstractAdults 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. Moreover, 13/41 had clinical complications of SCD, five requiring hospitalization.
Funder
Fondazione Città della Speranza