Guidance on the diagnosis and management of platelet-type von Willebrand disease: A communication from the Platelet Physiology Subcommittee of the ISTH.
GPIb alpha
PT-VWD
macrothrombocytopenia
platelet dysfunction
thrombocytopenia
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:
08 2020
08 2020
Historique:
received:
25
02
2020
revised:
21
03
2020
accepted:
30
03
2020
pubmed:
13
4
2020
medline:
15
5
2021
entrez:
13
4
2020
Statut:
ppublish
Résumé
Platelet-type von Willebrand disease (PT-VWD) is a rare autosomal dominant platelet bleeding disorder, with 55 patients reported worldwide so far, probably frequently misdiagnosed. Currently, there are no clear guidelines for the diagnosis and management of PT-VWD and this may contribute to misdiagnosis and thus to inappropriate treatment of these patients. This report provides expert opinion-based consensus recommendations for the standardized diagnostic and management approach to PT-VWD. Tests essential in the diagnostic workup are platelet count and size, ristocetin-induced platelet agglutination with mixing studies, and sequencing of platelet GP1BA gene. Platelet transfusions and von Willebrand factor-rich concentrates (if VWF is low) are the most effective treatments. This consensus may help to avoid misdiagnosis and guide appropriate management of patients with this disease.
Identifiants
pubmed: 32279414
doi: 10.1111/jth.14827
pii: S1538-7836(22)01591-4
doi:
Substances chimiques
Platelet Glycoprotein GPIb-IX Complex
0
von Willebrand Factor
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1855-1858Commentaires et corrections
Type : CommentIn
Type : CommentIn
Informations de copyright
© 2020 International Society on Thrombosis and Haemostasis.
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