Which type of von Willebrand disease (vWD) is associated with thrombocytopenia?

By William Aird

Type 2B vWD (gain-of-function defect in VWF that causes enhanced VWF–platelet interactions via platelet GPIb).

  • In type 2B VWD, VWF-GPIb platelet binding is pathologically enhanced, resulting in abnormal complexes between platelets and large adhesive forms of VWF. These complexes, not seen in other subtypes of VWD, are thought to account for the thrombocytopenia and depletion of large VWF multimers observed in many, but not all, cases of type 2B VWD.
  • The degree of thrombocytopenia can vary and may be exacerbated at times of increased VWF production or secretion, such as during physical effort, inflammation, or pregnancy.
  • Thrombocytopenia is rarely so severe as to be thought to contribute to clinical bleeding.
  • Thrombocytopenia, while a distinctive feature of type 2B VWD, is not always present at baseline.

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