Clinical, laboratory and therapeutic aspects of platelet-type von Willebrand disease

Int J Lab Hematol. 2008 Apr;30(2):91-4. doi: 10.1111/j.1751-553X.2007.00978.x.

Abstract

Platelet-type von Willebrand disease (PT-VWD), or pseudo-VWD, is a rare inherited platelet disorder characterized by an increased affinity of the platelet membrane glycoprotein Ibalpha receptor for normal von Willebrand factor leading to characteristic platelet hyperaggregability. As PT-VWD shares most of the clinical and laboratory features of subtype 2B VWD, the differential diagnosis between these two inherited bleeding disorders requires either platelet-mixing or molecular genetic studies. In this review, the main clinical, laboratory and therapeutic characteristics of PT-VWD are concisely reported.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Mutation
  • Platelet Aggregation
  • Platelet Glycoprotein GPIb-IX Complex / chemistry
  • Platelet Glycoprotein GPIb-IX Complex / genetics
  • Platelet Glycoprotein GPIb-IX Complex / metabolism
  • von Willebrand Diseases / blood
  • von Willebrand Diseases / classification
  • von Willebrand Diseases / diagnosis*
  • von Willebrand Diseases / therapy*
  • von Willebrand Factor / chemistry
  • von Willebrand Factor / genetics
  • von Willebrand Factor / metabolism

Substances

  • Platelet Glycoprotein GPIb-IX Complex
  • von Willebrand Factor