ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso-occlusive crisis of sickle cell disease.
ADAMTS13
sickle cell disease
thrombosis
vaso‐occlusion
von Willebrand factor
Journal
Research and practice in thrombosis and haemostasis
ISSN: 2475-0379
Titre abrégé: Res Pract Thromb Haemost
Pays: United States
ID NLM: 101703775
Informations de publication
Date de publication:
Jan 2021
Jan 2021
Historique:
received:
25
08
2020
revised:
29
10
2020
accepted:
05
11
2020
entrez:
4
2
2021
pubmed:
5
2
2021
medline:
5
2
2021
Statut:
epublish
Résumé
Sickle cell disease (SCD) is characterized by vaso-occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD-related endothelial injury. To decipher the role of VWF and its specific-cleaving metalloprotease, ADAMTS13, in the vaso-occlusive and thrombotic process of SCD. We investigated the VWF antigen (Ag), ADAMTS13 activity, ADAMTS13 Ag and ADAMTS13 IgGs in a cohort of 65 patients with SCD prospectively enrolled in a 20-month period from three centers. Patients were divided into two groups: an asymptomatic group (n = 30) with treated or untreated SCD at steady state, and a VOC/ACS group (n = 35) with SCD with VOC/ACS requiring either medical management or intensive care management for MOF. VWF:Ag levels were increased (median, 167 IU/dL; interquartile range [IQR], 124 - 279), especially in patients with VOC SCD (227 IU/dL; IQR, 134-305;
Sections du résumé
BACKGROUND
BACKGROUND
Sickle cell disease (SCD) is characterized by vaso-occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD-related endothelial injury.
OBJECTIVES
OBJECTIVE
To decipher the role of VWF and its specific-cleaving metalloprotease, ADAMTS13, in the vaso-occlusive and thrombotic process of SCD.
PATIENTS/METHODS
METHODS
We investigated the VWF antigen (Ag), ADAMTS13 activity, ADAMTS13 Ag and ADAMTS13 IgGs in a cohort of 65 patients with SCD prospectively enrolled in a 20-month period from three centers. Patients were divided into two groups: an asymptomatic group (n = 30) with treated or untreated SCD at steady state, and a VOC/ACS group (n = 35) with SCD with VOC/ACS requiring either medical management or intensive care management for MOF.
RESULTS AND CONCLUSIONS
CONCLUSIONS
VWF:Ag levels were increased (median, 167 IU/dL; interquartile range [IQR], 124 - 279), especially in patients with VOC SCD (227 IU/dL; IQR, 134-305;
Identifiants
pubmed: 33537544
doi: 10.1002/rth2.12460
pii: S2475-0379(22)01311-5
pmc: PMC7845082
doi:
Types de publication
Journal Article
Langues
eng
Pagination
197-203Informations de copyright
© 2020 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis (ISTH).
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