19. Pathophysiology, Epidemiology, Diagnosis, and Treatment of Acquired von Willebrand Syndrome

  1. Augusto B. Federici MD3,
  2. Christine A. Lee MA, MD, DSc (Med), FRCP, FRCPath, FRCOGad eundem4,
  3. Erik E. Berntorp MD, PhD5,
  4. David Lillicrap MD6 and
  5. Robert R. Montgomery MD7,8
  1. Ulrich Budde MD1,
  2. Augusto B. Federici MD3 and
  3. Jacob H. Rand2

Published Online: 21 MAR 2011

DOI: 10.1002/9781444329926.ch19

Von Willebrand Disease: Basic and Clinical Aspects

Von Willebrand Disease: Basic and Clinical Aspects

How to Cite

Budde, U., Federici, A. B. and Rand, J. H. (2011) Pathophysiology, Epidemiology, Diagnosis, and Treatment of Acquired von Willebrand Syndrome, in Von Willebrand Disease: Basic and Clinical Aspects (eds A. B. Federici, C. A. Lee, E. E. Berntorp, D. Lillicrap and R. R. Montgomery), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444329926.ch19

Editor Information

  1. 3

    Division of Hematology and Transfusion Medicine, L. Sacco University Hospital, Department of Internal Medicine, University of Milan, Milan, Italy

  2. 4

    University of London, London, UK

  3. 5

    Malmö Centre for Thrombosis and Haemostasis, Lund University, Skåne University Hospital, Malmö, Sweden

  4. 6

    Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Kingston, ON, Canada

  5. 7

    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA

  6. 8

    Blood Research Institute, Blood Center of Wisconsin, Milwaukee, WI, USA

Author Information

  1. 1

    Department of Hemostaseology, Medilys Laborgesellschaft mbH, c/o Asklepios Klinik Altona, Hamburg, Germany

  2. 2

    Hematology Laboratory, Department of Pathology, Montefiore Center, Bronx, NY, USA

  3. 3

    Division of Hematology and Transfusion Medicine, L. Sacco University Hospital, Department of Internal Medicine, University of Milan, Milan, Italy

Publication History

  1. Published Online: 21 MAR 2011
  2. Published Print: 25 MAR 2011

ISBN Information

Print ISBN: 9781405195126

Online ISBN: 9781444329926

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Keywords:

  • acquired von Willebrand syndrome;
  • inhibitors;
  • lymphoproliferative diseases;
  • cardiovascular diseases;
  • thrombocythemia;
  • autommune conditions

Summary

An acquired bleeding disorder with clinical and laboratory features similar to inherited von Willebrand disease (VWD) has been reported since 1968 and has been considered to be very rare. It was defined as acquired von Willebrand syndrome (aVWS) because it is similar to VWD and occurs late in life in patients with a negative history of bleeding who suffer mainly from lymphoproliferative, cardiovascular, and myeloproliferative disorders. In most instances aVWS is identified because of bleeding complications, and more than 80% of the patients are active bleeders. New bleeds after the diagnosis has been established occur in about 20% of patients, 33% of whom can require surgery within 1 year of diagnosis. Owing to the high risk of new bleeds and the necessity for surgery in many patients, it is important to establish a firm diagnosis and implement appropriate treatment in these patients.