Orphanet: Von Willebrand disease type 3

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Von Willebrand disease type 3

Disease definition

Type 3 von Willebrand disease (type 3 VWD) is the most severe form of VWD (see this term) characterized by a bleeding disorder associated with a total or near-total absence of Willebrand factor (von Willebrand factor; VWF) in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII (FVIII).


Classification level: Subtype of disorder
  • Synonym(s): -
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: D68.0
  • OMIM: 277480
  • UMLS: C1264041
  • MeSH: D056729
  • GARD: -
  • MedDRA: -

Detailed information

Article for general public


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