Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. Review uri icon

Overview

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.

publication date

  • February 8, 2013

Research

keywords

  • von Willebrand Diseases

Identity

Scopus Document Identifier

  • 84874190092

Digital Object Identifier (DOI)

  • 10.1055/s-0033-1334867

PubMed ID

  • 23397553

Additional Document Info

volume

  • 39

issue

  • 2