Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: A 2013 update

Augustob Federici, Ulrich Budde, Giancarlo Castaman, Jacobh Rand, Andreas Tiede

Research output: Contribution to journalArticlepeer-review

111 Scopus citations

Abstract

Acquired von Willebrand syndrome (AVWS) is an acquired bleeding disorder, first reported in 1968, with clinical and laboratory features similar to inherited von Willebrand disease. This rare bleeding disorder occurs mainly in patients with underlying lymphoproliferative, cardiovascular, myeloproliferative, and immunologic disorders. In contrast to acquired hemophilia A, AVWS is rarely associated with measurable anti-von Willebrand factor inhibitors. In most instances, AVWS is identified because of bleeding complications: in fact, more than 80% of the patients with this syndrome are active bleeders. Recurrent bleeding episodes occur in approximately 20 to 33% of patients with AVWS, especially following major trauma and surgery. Because of the heterogeneous mechanisms of AVWS, more than one therapeutic approach is often required to prevent or treat acute bleedings. Remission from some forms of AVWS can be obtained when the underlying disorders are treated.

Original languageEnglish
Pages (from-to)191-201
Number of pages11
JournalSeminars in Thrombosis and Hemostasis
Volume39
Issue number2
DOIs
StatePublished - 2013
Externally publishedYes

Keywords

  • acquired von Willebrand syndrome
  • autoimmune conditions
  • cardiovascular diseases
  • inhibitors
  • lymphoproliferative diseases
  • thrombocythemia

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