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Von Willebrand disease

Disease definition

A rare, inherited bleeding disorder characterized by defective platelet adhesion and secondary coagulation defect that manifests as abnormal bleeding of variable severity occurring either spontaneously or in association with an invasive procedure. Three main subtypes are defined based on the type of von Willebrand factor defect: partial (type 1) or total (type 3) deficiency, and qualitative/functional anomalies (type 2).


Classification level: Disorder
  • Synonym(s):
    • Hereditary von Willebrand disease
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: All ages
  • ICD-10: D68.0
  • ICD-11: 3B12
  • OMIM: 193400  277480  314560  613554
  • UMLS: C0042974
  • MeSH: D014842
  • GARD: -
  • MedDRA: 10047715

Detailed information

Article for general public


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