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von Willebrand Disease

  • James P. R. BrownEmail author
  • Joanne Douglas
Chapter

Abstract

This chapter outlines the pathophysiology, clinical presentation, inheritance, subclassification, and incidence of von Willebrand disease. The chapter describes the interaction with pregnancy and discusses the medical and anesthetic management of pregnant women with von Willebrand disease, including treatment of acute hemorrhage.

Keywords

von Willebrand disease Inherited bleeding disorder Factor VIII Desmopressin Inherited coagulopathy 

References

  1. 1.
    Bolton-Maggs P, Lillicrap D, Goudemand J, Berntorp E. von Willebrand disease update: diagnosis and treatment dilemas. Haemophilia. 2008;14:56–61.CrossRefGoogle Scholar
  2. 2.
    Katz D, Beilin Y. Disorders of coagulation in pregnancy. Br J Anaesth. 2015;115:ii75–88.CrossRefGoogle Scholar
  3. 3.
    American Society of Hematology. Quick reference: 2012 clinical practice guideline on the evaluation and management of von Willebrand Disease (VWD). 2012. Adapted from: The diagnosis, evaluation, and management of von Willebrand disease. National Heart, Lung, and Blood Institute, NIH Pub no 08-5832. Dec 2007.Google Scholar
  4. 4.
    Chi C, Kadir R. Inherited bleeding disorders in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2012;26:103–17.CrossRefGoogle Scholar
  5. 5.
    Bolton-Maggs P, Favaloro E, Hillarp A, Jennings I, Kohler H. Difficulties and pitfalls in the laboratory diagnosis of bleeding disorders. Haemophilia. 2012;18:66–72.CrossRefGoogle Scholar
  6. 6.
    The Australian Haemophilia Centre Directors’ Organisation. Evidence-based clinical practice guidelines for the use of recombinant and plasma-derived FVIII and FIX products. 2006 National Blood Authority: Australian Health Ministers’ Advisory Council. http://www.ahcdo.org.au/sitebuilder/publications/knowledge/asset/files/1/finalglswithbookmarksjune2006.pdf. Accessed 4 May 2016.
  7. 7.
    Varughese J, Cohen A. Experience with epidural anaesthesia in pregnant women with von Willebrand disease. Haemophilia. 2007;13:730–3.CrossRefGoogle Scholar
  8. 8.
    Chi C, Lee C, England A, Hingorani J, Paintsil J, Kadir R. Obstetric analgesia and anaesthesia in women with inherited bleeding disorders. Thromb Haemost. 2009;101:1104–11.CrossRefGoogle Scholar
  9. 9.
    Choi S, Brull R. Neuraxial techniques in obstetric and non-obstetric patients with common bleeding diatheses. Anesth Analg. 2009;109:648–60.CrossRefGoogle Scholar
  10. 10.
    Amorde R, Patel S, Pagel P. Mangement of labor and delivery of a patient with von Willebrand disease type 2A. Int Anesthesiol Clin. 2011;49:74–80.CrossRefGoogle Scholar
  11. 11.
    Jones B, Bell E, Maroof M. Epidural labor analgesia in a parturient with von Willebrand's disease type IIA and severe preeclampsia. Anesthesiology. 1999;90:1219–20.CrossRefGoogle Scholar
  12. 12.
    Caliezi C, Tsakiris D, Behringer H, Kuhne T, Marbet G. Two consecutive pregnancies and deliveries in a patient with von Willebrand's disease type 3. Haemophilia. 1998;4:845–9.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.UBC Department of Anesthesiology, Pharmacology and TherapeuticsBC Women’s HospitalVancouverCanada
  2. 2.Department of AnesthesiaBC Women’s HospitalVancouverCanada

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