Von Willebrand disease

D68.0

• Description
• Diagnositc criteria
• General and supportive measures
• Medicine treatment
• Referral

DESCRIPTION

Von Willebrand disease is the most common congenital bleeding disorder and is due to reduced amounts or abnormal forms of von Willebrand factor in the circulation.

DIAGNOSTIC CRITERIA

Clinical

• Recurrent epistaxis, prolonged bleeding from lacerations, easy bruising or gum bleeds.

Investigations

• Reduction in one or more of the following:
  • von Willebrand factor antigen,
  • ristocetin co-factor and/or collagen binding activity,
  • factor VIII coagulant activity.

GENERAL AND SUPPORTIVE MEASURES

• Apply pressure to the bleeding site.
• For tooth socket bleeds bite down on a piece of gauze.
• For epistaxis, see Chapter – Ear, nose, throat: Epistaxis (Nose bleeds) .
  

Avoid aspirin and NSAIDS.

MEDICINE TREATMENT

For bleeds:

• Factor VIII, IV (Factor VIII containing von Willebrand factor).
  • Initial dose: 30 units/kg.

For mucous membrane bleeds:

• Tranexamic acid, oral, 25 mg/kg/dose 6–8 hourly.

For menorrhagia:

• Combined oral contraceptive, low dose.

REFERRAL

• All suspected cases of von Willebrand disease to a haemophilia treatment centre for assessment.