Canis ISSN: 2398-2942

Von Willebrand's disease

Synonym(s): VWD

Contributor(s): Prof Bernard Feldman, Clare Knottenbelt, Mayank Seth

Introduction

  • Cause: typically an inherited disorder.
  • Acquired forms well recognized in man due to a variety of primary diseases, including cardiac disease and neoplasia. Described in dogs with mitral valve disease.
  • Deficiency or dysfunction of Von Willebrand factor (VWF) sometimes known as Factor VIII related antigen.
  • Partial or absolute lack of VWF  +/- VWF dysfunction → decreased platelet aggregation and adhesion (primary hemostatic defect).
  • Signs: range of symptoms from sub-clinical to life threatening hemorrhage, usually secondary to surgery or trauma.
  • Treatment: transfusion with plasma, cryoprecititate or fresh whole blood. Desmopressin (DDAVP) may have some beneficial effect.
  • Prognosis: incurable, problems are usually mild and inconsequential, risk of life-threatening hemorrhage.
    Print off the owner factsheet on Von Willebrand's disease (VWD) von Willebrand's disease (VWD) to give to your client.

Pathogenesis

Etiology

  • VWF is glycoprotein, produced by canine endothelial cells and megakaryocytes.
  • Acts as linking molecule facilitating platelet aggregation and adhesion to exposed vascular subendothelium.
  • Partial or absolute deficiency of Von Willebrand factor → defective primary hemostasis.
  • Three different types are recognized in dogs:
    • Type I:
      • Commonest type.
      • Associated with a partial lack of VWF.
      • Typically mildest manifestation.
      • Autosomal dominant inheritance with incomplete penetrance.
    • Type II:
      • Associated with a partial lack of VWF.
      • Also associated with decreased molecule size, limiting adhesive properties (VWF dysfunction).
      • More severe clinical manifestation possible.
      • Autosomal recessive inheritance.
    • Type III:
      • Associated with absence of VWF.
      • Typically severe clinical manifestation.
      • Autosomal recessive inheritance.

Predisposing factors

General
  • Concurrent disease that impair platelet function may exacerbate development of clinical signs:
    • Renal disease.
    • Hepatic disease.
    • Cardiac disease.
    Association with hypothyroidism has been disproved (Panciera, 1997).

Specific

Pathophysiology

  • May be rarely acquired - associated with underlying disease (cardiac disease, neoplasia, others) but usually autosomal inherited condition.

Timecourse

  • Acute in severe cases:
    • Type II/III cases often manifest as neonates.
  • In mild cases may remain subclinical for many years:
    • Type I cases may have excessive bleeding associated with loss of deciduous teeth or neutering but may go unnoticed.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Callan M B, Giger U, Catalfamo J L (2005) Effect of desmopressin on von Willebrand factor multimers in Doberman Pinschers with type I von Willebrand disease. Am J Vet Res 66 (5), 861-867 PubMed.
  • Venta P J, Li J, Yuzbasiyan-Gurkan V et al (2000) Mutation causing von Willebrand's disease in Scottish Terriers. J Vet Intern Med 14 (1), 10-9 PubMed.
  • de Gopegui R R & Feldman B F (1998) Acquired and inherited platelet dysfunction in small animals. Com Cont Educ Pract Vet 20 (9), 1039-1052 VetMedResource.
  • de Gopegui R R & Feldman B F (1997) Von Willebrand's disease. Comp Haem Int 7 (4), 187-196 SpringerLink.
  • Panciera D L (1997) Does abnormal hemostasis occur in canine hypothyroidism? Canine Prac 22 (1), 31-32 VetMedResource.
  • Thomas J S (1996) Von Willebrand's disease in the dog and cat. Vet Clin North Am 26 (5), 1089-1110 PubMed.
  • Brooks M, Dodds W J & Raymond S L (1992) Epidemiological features of von Willebrand's disease in Doberman Pinschers and Shetland sheepdogs - 200 cases (1984-1988). JAVMA 200 (8), 1123-1127 PubMed.

Other sources of information

  • Brooks M B & Catalfamo J L (2010) Von Willebrand Disease. In: Schalm’s Veterinary Hematology. 6th edn. Weiss D J & Wardrop K J. Wiley Blackwell. pp 612-618.
  • Carr A P, Nibblett B M & Panciera D L (2009) von Willebrand's Disease and Other Hereditary Coagulopathies. In: Kirks' Current Veterinary Therapy XIV. Bonagura J C & Twedt D C (eds), Philadelphia: W B Saunders. pp 277-280.
  • Ruiz de Gupegui R & Feldman B F (1999) Platelets and von Willebrand's disease. In: Textbook of Veterinary Internal Medicine. Ettinger S J & Feldman B F (Eds). Philadelphia: W B Saunders. pp 1816-1828
  • Reagan W J & Rebar A H (1995) Platelet disorders. In: Textbook of Internal Medicine.Ettinger S J & Feldman E C (Eds). London: W B Saunders. pp 1973-1974.
  • For information on Genetic Testing Providers (labs) genetic tests, and tests by breed, visit International Partnership for Dogs (https://dogwellnet.com/).
  • For a list of DNA tests available for certain breeds worldwide, visit Kennel Club Worldwide DNA tests: https://www.thekennelclub.org.uk/health/for-breeders/dna-testing-simple-inherited-disorders/worldwide-dna-tests/.


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