What is von Willebrand disease?
Von Willebrand disease (vWD) is a hereditary haemostasis disorder caused by a quantitative or qualitative deficiency of von Willebrand factor (vWF) β a protein that bridges platelets to subendothelial collagen during primary haemostasis. Without sufficient functional vWF, minor bleeds (cuts, surgical wounds, whelping) do not stop normally.
Types
- Type 1 (most common): mild-moderate quantitative vWF deficiency. Most dogs only bleed excessively under haemostatic stress (surgery, trauma). Breeds: Dobermann (up to 70 % of the breed are carriers), Poodle, Scottish Terrier, Shetland Sheepdog.
- Type 2: qualitative deficiency. Moderate to severe bleeding. German Shorthaired Pointer, German Wirehaired Pointer.
- Type 3: near-total absence of vWF. Severe clinical bleeding. Scottish Terrier, Chesapeake Bay Retriever, Kooikerhondje.
Symptoms
- Spontaneous mucosal bleeding: gums, nose (epistaxis), urinary tract (haematuria), gastrointestinal tract.
- Excessive or prolonged bleeding after cuts, dental extractions or surgery.
- Spontaneous bruising.
- In females: prolonged bleeding during oestrus or whelping.
- Many Type 1 dogs are asymptomatic until a surgical procedure.
Diagnosis
- von Willebrand factor antigen (vWF:Ag): the reference test. Normal >70 %; <50 % = increased risk; <35 % = high risk.
- DNA test: available for Types 1 and 3 in specific breeds (Dobermann, Scottish Terrier). Identifies clear, carrier and affected dogs before breeding decisions.
Treatment
Pre-surgical preparation
- Desmopressin (DDAVP): 1 ΞΌg/kg IV or intranasal 30β60 min before surgery. Releases endogenous vWF from endothelial cells. Effective in mild Type 1 for several hours.
- Fresh frozen plasma or cryoprecipitate: provides exogenous vWF; indicated for Types 2β3 or when desmopressin is insufficient. Cryoprecipitate is the treatment of choice for active severe bleeding.
Responsible breeding
DNA testing for vWD (available for Dobermanns and other breeds) identifies clear, carrier and affected dogs. Mating clear Γ clear eliminates the disease from offspring. Carriers can be bred to clear dogs (50 % carriers, 0 % affected). Type 2 and 3 affected dogs should not be bred.
