Von Willebrand disease type 1 (VWD1)
Von Willebrand disease type 1 (vWD1) is a bleeding disorder of variable severity that results from a quantitative or qualitative defect in von Willebrand factor (vWF). On vascular injury, vWf mediates platelet adhesion to exposed subendothelium and is involved in platelet-to-platelet aggregation. The disease is the most common hereditary bleeding disorder and it is genetically and clinically heterogeneous. Clinical signs of the disease include spontaneous bleeding from mucosal surfaces and excess blood loss after surgery or trauma. Three clinical types, 1, 2, and 3, have been described.
Von Willebrand disease type 1 is inherited in a recessive fashion. Bleeding appears to be due to the reduced amount of vWF rather than a qualitative difference. Although Von Willebrand disease type 1 is the most common form of vWD found in most mammals and can cause serious bleeding problems, it is generally less severe than the other 2 types.
Testing of affected dogs can be done by vWF antigen testing or by coagulation assays, but these procedures yield variable results. This variability makes it difficult for breeders to use this information to eliminate the disease-causing allele from their lines. Thus, it is highly desirable that DNA tests are run to get definitive answers.
Moser, J., Meyers, K.M., Meinkoth, J.H., and Brassard, J.A. (1996). Temporal variation and factors affecting measurement of canine von Willebrand factor. American Journal of Veterinary Research 57, 1288–1293.