Lymphoma-associated acquired von Willebrand syndrome responsive to splenectomy: A case report.
acquired von Willebrand syndrome
case report
hemostasis
Journal
EJHaem
ISSN: 2688-6146
Titre abrégé: EJHaem
Pays: United States
ID NLM: 101761942
Informations de publication
Date de publication:
Aug 2022
Aug 2022
Historique:
received:
28
04
2022
revised:
05
05
2022
accepted:
12
05
2022
entrez:
2
9
2022
pubmed:
3
9
2022
medline:
3
9
2022
Statut:
epublish
Résumé
A previously healthy 33-year-old female presented with a large hematoma over her right knee after kneeling. She was found to have pancytopenia and massive splenomegaly. Von Willebrand Factor (VWF) antigen level was 0.38 units/ml, ristocetin cofactor activity 0.13 units/ml, and VWF multimeric distribution was normal. Bone marrow examination revealed an indolent B-cell lymphoma. Diagnosis was consistent with acquired von Willebrand syndrome as an autoimmune epiphenomenon of a lymphoma. Diagnostic and therapeutic splenectomy under hemostatic coverage was performed. VWF antigen levels and activities immediately normalized postoperatively and remained within the normal range several months later. Splenic pathology confirmed hairy cell leukemia with a BRAF mutation.
Identifiants
pubmed: 36051021
doi: 10.1002/jha2.486
pii: JHA2486
pmc: PMC9421994
doi:
Types de publication
Journal Article
Langues
eng
Pagination
996-999Informations de copyright
© 2022 The Authors. eJHaem published by British Society for Haematology and John Wiley & Sons Ltd.
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