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

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

Giulia Reggiani  1 , Maria Paola Boaro  1 , Federica Menzato  1 , Emiliano De Bon  2 , Antonella Bertomoro  3 , Alessandra Casonato  3 , Fabrizio Lucente  3 , Cecilia Fortino  3 , MariaTeresa Sartori  3 , Agostino Steffan  4 , Maddalena Martella  1 , Mirco D'Agnolo  1 , Laura Sainati  1 , Raffaella Colombatti  1
Affiliations

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

Giulia Reggiani et al. Pediatr Blood Cancer. 2024 Jun.

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.

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References

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