3B12

ICD-11 MMS 2026-01

Von Willebrand disease

Description

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.

Index terms

  • Von Willebrand disease
  • Angiohaemophilia
  • Factor VIII deficiency with vascular defect
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  • Minot-von Willebrand-Jurgen disease
  • Vascular haemophilia
  • Willebrand Jurgen thrombopathy
  • angiohaemophilia A
  • angiohaemophilia B
  • pseudohaemophilia
  • Von Willebrand disease type 1
  • Von Willebrand disease type 2
  • Von Willebrand disease type 2A
  • Von Willebrand disease type 2B
  • Von Willebrand disease type 2M
  • Von Willebrand disease type 2N
  • Von Willebrand disease type 3

Inclusions

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

Excludes

  • 3B10 – factor VIII deficiency with functional defect
  • 3B2Y – Acquired von Willebrand disease or syndrome

Exclusions from above levels

  • JA43.3 – Postpartum coagulation defects
  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth or the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3B10 – Hereditary factor VIII deficiency
  • 3B11 – Hereditary factor IX deficiency
  • 3B13 – Haemophilia C
  • 3B14 – Other inherited coagulation factor deficiency with bleeding tendency
  • 3B15 – Inherited coagulation factor deficiency without bleeding tendency
  • 3B1Z – Congenital or constitutional haemorrhagic condition, unspecified

Foundation entity

Von Willebrand disease – http://id.who.int/icd/entity/2112021600

Part of: 03: Diseases of the blood or blood-forming organs