Longitudinal bleeding assessment in von Willebrand disease utilizing an interim bleeding score.
hemorrhage
phenotype
symptom assessment
von Willebrand disease
von Willebrand factor
Journal
Journal of thrombosis and haemostasis : JTH
ISSN: 1538-7836
Titre abrégé: J Thromb Haemost
Pays: England
ID NLM: 101170508
Informations de publication
Date de publication:
10 2022
10 2022
Historique:
revised:
24
05
2022
received:
17
01
2022
accepted:
10
06
2022
pubmed:
4
7
2022
medline:
23
9
2022
entrez:
3
7
2022
Statut:
ppublish
Résumé
Assessment of bleeding phenotype is critically important in the diagnosis of von Willebrand disease (VWD). Despite advances in bleeding assessment tools (BATs), standardized tools to evaluate bleeding following diagnosis (interim bleeding) are lacking. We assessed the clinical utility of an interim bleeding protocol in a multicenter, international study involving patients with VWD. The enrolment ISTH BAT formed the original bleeding score (0 BS). At follow-up, the International Society on Thrombosis and Haemostasis BAT was repeated but included only interval bleeding (Interim BS, 1 BS). Both scores were annualized (0 BS/yr, 1 BS/yr). BS were analyzed by VWD subtype, plasma VWF level, sex, and age. Interim BS discriminated by subtype, with significantly increased 0 BS and 1 BS in patients with type 3 VWD. In patients with type 1 VWD, a positive or negative 0 BS did not predict future bleeding, with similar 1 BS/yr (median 1.0 vs. 0.7, p = .2). Despite significantly higher 0 BS in females with type 1 VWD than males (median 7 vs. 5, p = .0012), 1 BS were not significantly different (median 4 vs. 4, p = .16). While 0 BS were lower in children than adults with type 1 VWD, interim BS were similar (median 5 vs. 3, p = .5; 1BS/yr, median 1 vs. 0.8, p = .7). Interestingly, in those with plasma von Willebrand factor:ristocetin cofactor levels >50 IU/dl, interim BS rates were similar to those 30-50 IU/dl (1 BS/yr 0.8 vs. 1.3, p = .5). This study provides both a new approach to longitudinal bleeding assessment and insights into the evolution of bleeding in VWD.
Sections du résumé
BACKGROUND
Assessment of bleeding phenotype is critically important in the diagnosis of von Willebrand disease (VWD). Despite advances in bleeding assessment tools (BATs), standardized tools to evaluate bleeding following diagnosis (interim bleeding) are lacking.
OBJECTIVES
We assessed the clinical utility of an interim bleeding protocol in a multicenter, international study involving patients with VWD.
METHODS
The enrolment ISTH BAT formed the original bleeding score (0 BS). At follow-up, the International Society on Thrombosis and Haemostasis BAT was repeated but included only interval bleeding (Interim BS, 1 BS). Both scores were annualized (0 BS/yr, 1 BS/yr). BS were analyzed by VWD subtype, plasma VWF level, sex, and age.
RESULTS
Interim BS discriminated by subtype, with significantly increased 0 BS and 1 BS in patients with type 3 VWD. In patients with type 1 VWD, a positive or negative 0 BS did not predict future bleeding, with similar 1 BS/yr (median 1.0 vs. 0.7, p = .2). Despite significantly higher 0 BS in females with type 1 VWD than males (median 7 vs. 5, p = .0012), 1 BS were not significantly different (median 4 vs. 4, p = .16). While 0 BS were lower in children than adults with type 1 VWD, interim BS were similar (median 5 vs. 3, p = .5; 1BS/yr, median 1 vs. 0.8, p = .7). Interestingly, in those with plasma von Willebrand factor:ristocetin cofactor levels >50 IU/dl, interim BS rates were similar to those 30-50 IU/dl (1 BS/yr 0.8 vs. 1.3, p = .5).
CONCLUSION
This study provides both a new approach to longitudinal bleeding assessment and insights into the evolution of bleeding in VWD.
Identifiants
pubmed: 35780487
doi: 10.1111/jth.15807
pmc: PMC10193460
mid: NIHMS1893242
pii: S1538-7836(22)19041-0
doi:
Substances chimiques
von Willebrand Factor
0
Types de publication
Journal Article
Multicenter Study
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
2246-2254Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL112614
Pays : United States
Organisme : NCRR NIH HHS
ID : M01 RR000058
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL033721
Pays : United States
Organisme : NHLBI NIH HHS
ID : P01 HL144457
Pays : United States
Organisme : NHLBI NIH HHS
ID : P01 HL081588
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL139847
Pays : United States
Informations de copyright
© 2022 International Society on Thrombosis and Haemostasis.
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