von Willebrand Disease
To view the entire topic, please log in or purchase a subscription.
The Washington Manual of Medical Therapeutics helps you diagnose and treat hundreds of medical conditions. Consult clinical recommendations from a resource that has been trusted on the wards for 50+ years. Explore these free sample topics:
-- The first section of this topic is shown below --
General Principles
Classification
vWD has three main types1:
- Type 1 vWD, due to a quantitative deficiency of vWF (70%–80% of cases)
- Type 2 vWD, due to a qualitative defect of vWF, includes four subtypes (2A, 2B, 2M, 2N):
- Type 2A: reduced vWF high molecular weight multimer
- Type 2B: pathologically enhanced platelet affinity to vWF
- Type 2M: reduced platelet affinity to vWF
- Type 2N: defective FVIII binding to vWF
- Type 3 vWD, due to a near complete lack of vWF.2
Epidemiology
vWD, the most common inherited bleeding disorder, affects around 0.1%–1% of the population.
Etiology
Most forms of vWD have an autosomal dominant inheritance with variable penetrance, although autosomal recessive forms (types 2N and 3) exist. vWF circulates as multimers of variable size and facilitate adherence of platelets to injured vessel walls and stabilize FVIII in plasma.
-- To view the remaining sections of this topic, please log in or purchase a subscription --
General Principles
Classification
vWD has three main types1:
- Type 1 vWD, due to a quantitative deficiency of vWF (70%–80% of cases)
- Type 2 vWD, due to a qualitative defect of vWF, includes four subtypes (2A, 2B, 2M, 2N):
- Type 2A: reduced vWF high molecular weight multimer
- Type 2B: pathologically enhanced platelet affinity to vWF
- Type 2M: reduced platelet affinity to vWF
- Type 2N: defective FVIII binding to vWF
- Type 3 vWD, due to a near complete lack of vWF.2
Epidemiology
vWD, the most common inherited bleeding disorder, affects around 0.1%–1% of the population.
Etiology
Most forms of vWD have an autosomal dominant inheritance with variable penetrance, although autosomal recessive forms (types 2N and 3) exist. vWF circulates as multimers of variable size and facilitate adherence of platelets to injured vessel walls and stabilize FVIII in plasma.
There's more to see -- the rest of this entry is available only to subscribers.