ICD-11 classes
03 Diseases of the blood or blood-forming organs
Coagulation defects, purpura or other haemorrhagic or...
Coagulation defects
Congenital or constitutional haemorrhagic condition
3B12 — Von Willebrand disease

ICD-11 3B12 — Von Willebrand disease

A disease caused by inherited genetic mutations. This disease is characterised by quantitative, structural or function abnormalities of Von Willebrand factor leading to abnormalities in coagulation of the blood. This disease may present with prolonged bleeding, easy bruising or, bleeding gums. Confirmation is by identification of mutation through genetic testing.

It includes 6 items.

  • Factor VIII deficiency with vascular defect
  • Vascular haemophilia
  • Angiohaemophilia
  • Von Willebrand disease type 1
  • Von Willebrand disease type 2
  • Von Willebrand disease type 3

It excludes 3 items.

  • factor VIII deficiency with functional defect (3B10)
  • factor VIII deficiency NOS (3B10)
  • Acquired von Willebrand disease or syndrome (3B20-3B2Y)

It has no clarifying diagnoses.

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