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Acquired von Willebrand's disease

B J Hennessy1, B White, M Byrne

  • 1Department of Haemostasis and Thrombosis, St. James's Hospital, Dublin.

Irish Journal of Medical Science
|June 25, 1998
PubMed
Summary

Acquired von Willebrand

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Area of Science:

  • Hematology
  • Internal Medicine

Background:

  • Von Willebrand's disease (vWD) is a common inherited bleeding disorder.
  • Acquired von Willebrand's disease (AvWD) is a rare condition, often linked to autoimmune or clonal proliferation disorders.

Observation:

  • This study reports four cases of AvWD diagnosed over three years.
  • Patients lacked personal or family bleeding history, with AvWD confirmed by laboratory tests.
  • Associated conditions included Waldenstrom's Macroglobulinaemia, hypothyroidism, and MGUS.

Findings:

  • The acquired hemostatic defect resolved in three patients after treating their primary condition.
  • One patient required ongoing von Willebrand Factor replacement for bleeding control.
  • Mechanisms may involve antibodies inactivating vWF or selective absorption by malignant cells.

Implications:

  • Effective management of AvWD can involve treating the underlying disorder.
  • Understanding AvWD mechanisms is crucial for developing targeted therapies.
  • These cases highlight the importance of considering AvWD in patients with unexplained bleeding and associated conditions.

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