Von Willebrand Disease (VWD)

The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Published: Aug 2014.

Article Last Updated:14/02/2026

Definition

VWD is the most common inherited bleeding disorder, characterised by the deficiency / dysfunction of von Willebrand factor (VWF).

VWF is a plasma glycoprotein involved in primary haemostasis (formation of a platelet plug), main functions are: ^[Ref]

Mediate platelet adhesion and aggregation at sites of vascular injury (VWF binds to exposed subendothelial collagen and to platelet glycoprotein Ibα)
Carrier and stabiliser for clotting factor VIII

Aetiology and Types

There are 3 types of VWD: ^[Ref]

Type	Prevalence	Definition	Inheritance pattern	Clinical severity
I	70-80%	Quantitative VWF deficiency	Autosomal dominant	Least severe
II	20%	Abnormal VWF (qualitative defect)	Autosomal dominant	Intermediate
III	<5%	Absence of VWF	Autosomal recessive	Most severe

Clinical Features

Presence of family history is typical.

VWD classically cause excessive mucocutaneous bleeding, including: ^[Ref]

Epistaxis (often frequent and prolonged)
Easy bruising, petechiae
Excessive bleeding from minor wounds
Spontaneous oral cavity bleeding (e.g. from teeth brushing)
Excessive bleeding after surgery / dental procedures / childbirth
Heavy menstrual bleeding (in women)

Musculoskeletal (intra-articular / muscular) bleeding is a classic feature seen in haemophilia, but not VWD. While mucocutaneous bleeding is characteristically seen in VWD, but not haemophilia.

This feature is helpful in differentiating between VWD and haemophilia in exams. However in practice, VWD can still cause musculoskeletal bleeding, esp. in those with type II/III disease.

Complications

Chronic bleeding can lead to iron deficiency anaemia.

Patients with VWD can develop angiodysplasia, which then predisposes them to GI bleeding.

Investigation and Diagnosis

Key laboratory findings in VWD:

Prolonged bleeding time (due to impaired platelet function)
Reduced VWF activity and antigen level
Reduced factor VIII activity (as VWF functions as a carrier and stabiliser for factor VIII)
Normal or prolonged APTT (due to reduced factor VIII activity)
- APTT can be normal if factor VIII activity is not significantly reduced (esp. in type I)
- Therefore, a normal APTT does NOT exclude the diagnosis

Importantly, the following parameters are normal in VWD:

Platelet count (only platelet function is impaired, but NOT platelet count)
PT (extrinsic clotting pathway is NOT affected, factor VIII is involved in the intrinsic clotting pathway – measured by APTT)

The diagnostic approach of specific types of VWD is omitted here, as it is complicated and deemed unnecessary for undergraduate level.

Apart from type 1, 2, and 3. There are another 4 subtypes of type 2 VWD.

Management

Long-Term Management

Offer a trial of desmopressin

MoA: desmopressin increases endogenous VWF and factor VIII levels
Treatment response should be assessed by measuring factor VIII activity, VWF antigen and VWF activity at baseline and after desmopressin administration
Desmopressin should be avoided in those with atherosclerosis

Exception: type III VWD is very severe will not respond to desmopressin, but its management is out of scope for undergraduate level.

If the patient suffers from heavy menstrual bleeding, also consider hormonal management (1st line: LNG-IUS, see the Heavy Menstrual Bleeding (HMB) article for more information)