What is type 1 von Willebrand disease (vWD)?

By William Aird

  • The most common type; accounts for about 60%-85% of cases.
  • The mildest form of disease.
  • Autosomal dominant inheritance.
  • Sequence variants absent in about 35% of patients.
  • Plasma vWF level < 30 units/dL, parallel reduction in factor VIII levels.
  • Variable degrees of bleeding.
  • Bleeding in type 1 vWD is caused by a decrease in concentration of vWF, not a selective reduction in large multimers. All multimers are present in decreased amounts.
  • Concordant reduction in VWF activity and antigen.
  • About 15% of cases (called type 1C) are associated with increased clearance of VWF.

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