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

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 s...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric blood & cancer 2024-06, Vol.71 (6), p.e30971-n/a
Main Authors: Reggiani, Giulia, Boaro, Maria Paola, Menzato, Federica, De Bon, Emiliano, Bertomoro, Antonella, Casonato, Alessandra, Lucente, Fabrizio, Fortino, Cecilia, Sartori, MariaTeresa, Steffan, Agostino, Martella, Maddalena, D'Agnolo, Mirco, Sainati, Laura, Colombatti, Raffaella
Format: Article
Language:English
Subjects:
ADAMTS13
Adolescent
Child
Child, Preschool
Children
coagulation
Coagulation factors
Cohort Studies
Female
Genotypes
Humans
Infant
Male
Plasminogen activator inhibitors
Sickle cell disease
sickle cell trait
Sickle Cell Trait - blood
Sickle Cell Trait - complications
t-Plasminogen activator
Thromboembolism
Thrombophilia - blood
Thrombophilia - etiology
Von Willebrand factor
von Willebrand Factor - analysis
von Willebrand Factor - metabolism
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.30971