Von Willebrand disease I

Other Names:
Pseudohemophilia, Vascular hemophilia, von Willebrand disease type 1, von Willebrand's disease, VWDI
Inheritance:
Autosomal Recessive
Mutation(s):
G>A
Affected Gene(s):
VWF
Affected Breed(s):
Aussiedoodle, Australian Labradoodle, Barbet, Bernedoodle, Bernese Mountain Dog, Brazilian Terrier, Cardigan Welsh Corgi, Cavapoo, Cockapoo, Coton de Tulear, Doberman Pinscher, Drentsche Patrijshond, Dutch Partridge Dog, Dutch Shepherd, French Water Dog, German Pinscher, Goldendoodle, Irish Red and White Setter, Irish Setter, Kerry Blue Terrier, Kromfohrländer, Labradoodle, Maltipoo, Manchester Terrier, Miniature Poodle, Newfypoo, Papillon, Pembroke Welsh Corgi, Phalene, Poodle, Stabyhoun, Standard Poodle, Toy Manchester Terrier, Toy Poodle, West Highland White Terrier

Common Symptoms

Von Willebrand’s disease type I (VWDI) is an inherited bleeding disorder affecting many breeds of dog. Dogs affected with VWDI have less than half of the normal level of von Willebrand coagulation factor (vWf), which is an essential protein needed for normal blood clotting. There is variability in the amount of vWf such that not all dogs with two copies of the mutation are equally affected. Dogs that have less than 35% of the normal amount of vWf generally have mild to moderate signs of a bleeding disorder. Affected dogs may bruise easily, have frequent nosebleeds, bleed from the mouth when juvenile teeth are lost, and experience prolonged bleeding after surgery or trauma. Less often, the bleeding may be severe enough to cause death. Due to the variable severity of the disorder, affected dogs may not be identified until a surgery is performed or trauma occurs at which time excessive bleeding is noted. Veterinarians performing surgery on known affected dogs should have ready access to blood banked for transfusions. Most dogs will have a normal lifespan with this condition despite increased blood clotting times.

References

  • Brooks MB, Erb HN, Foureman PA, Ray K. von Willebrand disease phenotype and von Willebrand factor marker genotype in Doberman Pinschers. Am J Vet Res. 2001 Mar;62(3):364-9. [PubMed: 11277201]
  • Shaffer LG, Ramirez CJ, Sundin K, Carl C, Ballif BC (2015) Genetic screening and mutation identification in a rare canine breed, the Drentsche patrijshond. Vet Rec Case Rep. 3:e000185.