Von Willebrand Disease
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10 code: D68.0 DESCRIPTION Von Willebrand disease is the most common congenital bleeding disorder and is due to reduced amounts or abnormal forms of von Willebrand factor. DIAGNOSTIC CRITERIA Clinical Recurrent epistaxis, prolonged bleeding from lacerations, easy bruising or gum bleeds, menorrhag
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Referral
ICD-10 code: D68.0
DESCRIPTION
Von Willebrand disease is the most common congenital bleeding disorder and is due to reduced amounts or abnormal forms of von Willebrand factor.
DIAGNOSTIC CRITERIA
Clinical
- Recurrent epistaxis, prolonged bleeding from lacerations, easy bruising or gum bleeds, menorrhagia.
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: Epistaxis (Nose bleeds).
Avoid aspirin and avoid NSAIDS.
MEDICINE TREATMENT
For bleeds:
- Factor VIII, IV (Factor VIII-containing von Willebrand factor).
- Initial dose: 50 units/kg.
For mucous membrane bleeds:
- Tranexamic acid, oral, 25 mg/kg/dose 6–8 hourly.
For menorrhagia:
- Combined oral contraceptive, low dose.
Also refer to Adult STGs and EML; Gynaecology, Uterine Bleeding, Abnormal.
REFERRAL
- All suspected cases of von Willebrand disease to a Haemophilia Treatment Centre for diagnostic assessment and treatment.
- Symptomatic thrombocytopenia.
Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_ Updated February 2025
Chapter 3: Blood and Blood Forming Organs, 3.7 Von Willebrand Disease