Successful use of lenalidomide to treat refractory acquired von Willebrand disease associated with monoclonal gammopathy.
Acquired von Willebrand syndrome
Dexamethasone
Lenalidomide
Monoclonal gammopathy of clinical significance
Monoclonal gammopathy of undetermined significance
Journal
Annals of hematology
ISSN: 1432-0584
Titre abrégé: Ann Hematol
Pays: Germany
ID NLM: 9107334
Informations de publication
Date de publication:
Dec 2022
Dec 2022
Historique:
received:
22
05
2022
accepted:
27
09
2022
pubmed:
5
10
2022
medline:
15
11
2022
entrez:
4
10
2022
Statut:
ppublish
Résumé
Acquired von Willebrand syndrome (AVWS) is a rare hematologic disorder characterized by quantitative or qualitative defects of von Willebrand factor (vWF), a protein crucial for normal hemostasis. AVWS has been described in association with several pathologic entities with varied mechanisms. Among these, lymphoproliferative disorders are the most common, with monoclonal gammopathy of undetermined significance (MGUS) being the most frequently reported. AVWS in this setting is commonly associated with the development of bleeding that is clinically challenging to manage due to accelerated clearance of vWF, limiting the utility of many conventional treatment modalities such as DDAVP or vWF/FVIII. We report a case of a 43-year-old male who was sent to our institution for new-onset easy bruising and laboratories concerning for von Willebrand disease (vWD). Further diagnostic workup revealed evidence of an IgG monoclonal gammopathy and findings suggestive of vWF inhibition. Ultimately, he was found to have monoclonal gammopathy of clinical significance (MGCS)-associated AVWS refractory to conventional treatment but responsive to lenalidomide and dexamethasone. This case suggests that lenalidomide may be suitable for patients with AVWS secondary to MGCS.
Identifiants
pubmed: 36194257
doi: 10.1007/s00277-022-04991-9
pii: 10.1007/s00277-022-04991-9
pmc: PMC9530414
doi:
Substances chimiques
von Willebrand Factor
0
Lenalidomide
F0P408N6V4
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
2627-2631Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
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