Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management

BMJ Case Rep. 2023 Dec 6;16(12):e257760. doi: 10.1136/bcr-2023-257760.

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.

Keywords: Haematology (incl blood transfusion); Malignant and Benign haematology; Medical management.

Publication types

  • Case Reports

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • von Willebrand Diseases* / diagnosis
  • von Willebrand Diseases* / etiology
  • von Willebrand Diseases* / therapy
  • von Willebrand Factor / therapeutic use

Substances

  • Immunoglobulins, Intravenous
  • von Willebrand Factor