A rare case of acquired von Willebrand syndrome type 2B: diagnosis, treatment, and underlying pathophysiology.
acquired von Willebrand syndrome
hemorrhage
monoclonal gammopathy
platelet glycoprotein GPIb
type 2B von Willebrand disease
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:
Jul 2024
Jul 2024
Historique:
received:
05
04
2024
revised:
14
06
2024
accepted:
18
06
2024
medline:
4
9
2024
pubmed:
4
9
2024
entrez:
4
9
2024
Statut:
epublish
Résumé
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that usually mimics type 1 or 2A von Willebrand disease (VWD). Can AVWS mimic the phenotype of type 2B VWD? A 64-year-old male patient presented with thrombocytopenia, normal routine hemostasis results, and normal VWF antigen and factor VIII levels but reduced von Willebrand factor (VWF) activity (31 IU/dL). The ristocetin-induced platelet aggregation test showed paradoxical aggregation at low doses of ristocetin, suggesting type 2B VWD, but no deleterious sequence variation was found in either the This AVWS with a 2B phenotype VWD was probably related to a monoclonal immunoglobulin G antibody causing a VWF conformational change, resulting in increased affinity to platelet glycoprotein-Ib. In the event of surgery or bleeding, treatment with vonicog alfa seems to be the best option for this patient.
Sections du résumé
Background
UNASSIGNED
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that usually mimics type 1 or 2A von Willebrand disease (VWD).
Key Clinical Question
UNASSIGNED
Can AVWS mimic the phenotype of type 2B VWD?
Clinical Approach
UNASSIGNED
A 64-year-old male patient presented with thrombocytopenia, normal routine hemostasis results, and normal VWF antigen and factor VIII levels but reduced von Willebrand factor (VWF) activity (31 IU/dL). The ristocetin-induced platelet aggregation test showed paradoxical aggregation at low doses of ristocetin, suggesting type 2B VWD, but no deleterious sequence variation was found in either the
Conclusion
UNASSIGNED
This AVWS with a 2B phenotype VWD was probably related to a monoclonal immunoglobulin G antibody causing a VWF conformational change, resulting in increased affinity to platelet glycoprotein-Ib. In the event of surgery or bleeding, treatment with vonicog alfa seems to be the best option for this patient.
Identifiants
pubmed: 39228434
doi: 10.1016/j.rpth.2024.102516
pii: S2475-0379(24)00211-5
pmc: PMC11369413
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Pagination
102516Informations de copyright
© 2024 The Author(s).