Early-onset indicators of a hypercoagulable state and clinical complications in a cohort of children with sickle cell trait

Pediatr Blood Cancer. 2024 Jun;71(6):e30971. doi: 10.1002/pbc.30971. Epub 2024 Mar 30.

Abstract

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. Moreover, 13/41 had clinical complications of SCD, five requiring hospitalization.

Keywords: ADAMTS13; children; coagulation; sickle cell disease; sickle cell trait; von Willebrand factor.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Female
  • Humans
  • Infant
  • Male
  • Sickle Cell Trait* / blood
  • Sickle Cell Trait* / complications
  • Thrombophilia* / blood
  • Thrombophilia* / etiology
  • von Willebrand Factor / analysis
  • von Willebrand Factor / metabolism

Substances

  • von Willebrand Factor