You are here

  • Home
  • Archive
  • Volume 16, Issue 12
  • Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management

Article Text

Article menu
Download PDFPDF
Haematology
Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management
  1. Katherine Ismei Zhou1,
  2. Sargam Kapoor2 and
  3. Oluwatoyosi Adefunke Onwuemene2
  1. 1Department of Medicine, Duke University Medical Center, Durham, North Carolina, USA
  2. 2Division of Hematology, Department of Medicine, Duke University Medical Center, Durham, North Carolina, USA
  1. Correspondence to Dr Oluwatoyosi Adefunke Onwuemene; toyosi.onwuemene{at}duke.edu

Abstract

Acquired von Willebrand syndrome (AVWS) is a rare disorder that is characterised by an acquired deficiency of von Willebrand factor. AVWS was suspected in a patient with type III von Willebrand disease (VWD) who did not respond to factor replacement therapy. Given the crucial implications for management, we describe this patient’s clinical presentation, diagnosis and periprocedural management. To facilitate pericardiocentesis, periprocedural management included steroids, intravenous immunoglobulin and factor replacement therapy. In other patients with suspected immune-mediated AVWS, a similar approach may be effective. This case also highlights the importance of distinguishing AVWS from inherited VWD.

  • Haematology (incl blood transfusion)
  • Malignant and Benign haematology
  • Medical management

https://doi.org/10.1136/bcr-2023-257760

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text

    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms and critical revision for important intellectual content: KIZ, SK and OAO. The following authors gave final approval of the manuscript: KIZ, SK and OAO.

    • Funding This stydy was funded by National Heart, Lung, and Blood Institute (5T32HL007057-48).

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.