von Willebrand Disease

von Willebrand Disease is a topic covered in the Washington Manual of Medical Therapeutics.

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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.

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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.

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