[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [General pathology](https://durgical.com/en/general-pathology/) › [Red cells and bleeding disorders](https://durgical.com/en/general-pathology/areas/red-cells/) › Von Willebrand disease

# Von Willebrand disease

- Lesion

The commonest inherited bleeding disorder: too little or faulty von Willebrand factor, so platelets stick poorly and factor VIII falls.

Updated Nov 12, 2026

## Key points

- Type 1 (about 70%): mild to moderate quantitative deficiency, autosomal dominant.
- Type 2 (about 25%): qualitative defects; type 2A lacks large and intermediate multimers.
- Type 3: autosomal recessive near-absence of vWF, factor VIII low, haemophilia-like bleeding.
- Ristocetin cofactor activity reduced; PTT may be prolonged because factor VIII falls.
- Desmopressin releases stored vWF and helps types 1 and some type 2.

## What it is

Von Willebrand factor (vWF) is a large multimeric protein made by endothelial cells and megakaryocytes. It glues platelets (through GPIb/IX) to exposed subendothelium, helps aggregation at high shear and carries and stabilizes factor VIII in plasma. Von Willebrand disease is the most common inherited bleeding disorder, usually autosomal dominant. It causes mucosal bleeding, wound bleeding and heavy menstrual bleeding, with normal platelet counts; in severe forms factor VIII also falls and deep bleeding appears.

## Step by step

- Endothelium stores vWF in Weibel–Palade bodies and secretes it into plasma and subendothelium.
- After injury, vWF binds collagen and captures platelets through GPIb/IX.
- vWF also binds factor VIII and prolongs its half-life in plasma.
- Too little vWF or abnormal multimers: platelets adhere poorly, causing mucosal bleeding.
- In type 3 and severe type 1, factor VIII is degraded quickly and coagulation fails too.

## Types of von Willebrand disease

| Type | Defect | Inheritance | Bleeding |
| --- | --- | --- | --- |
| [Type 1](https://durgical.com/en/conditions/von-willebrand-disease/) | Partial quantitative deficiency of normal vWF | Autosomal dominant | Mild to moderate, mucosal |
| [Type 2 (2A most common)](https://durgical.com/en/conditions/von-willebrand-disease/) | Qualitative: abnormal multimers or binding | Usually autosomal dominant | Mild to moderate |
| [Type 3](https://durgical.com/en/conditions/von-willebrand-disease/) | Almost no vWF; factor VIII low | Autosomal recessive | Severe, joints and muscles too |

## Clinical notes

- Nosebleeds, gum bleeding, prolonged bleeding from small cuts and heavy menstrual bleeding, often from menarche.
- Wide variability even within a family; vWF levels are lower in blood group O.

## In the lab

- vWF antigen, ristocetin cofactor activity, factor VIII activity, multimer analysis.

## Memory aid

Von Willebrand is the glue and the bodyguard: without it platelets do not stick and factor VIII does not last.

## Test yourself

Think of the answer, then tap the question to see it.

## What two jobs does von Willebrand factor perform?

It mediates platelet adhesion to subendothelium through GPIb/IX and carries and stabilizes factor VIII.

## Which type of von Willebrand disease is most common?

Type 1, a mild to moderate quantitative deficiency inherited as an autosomal dominant trait.

## Why can the PTT be prolonged in von Willebrand disease?

Low vWF shortens the survival of factor VIII, lowering its level.

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## Elsewhere in DurgiPedia

- [Primary hemostasis and platelets](https://durgical.com/en/physiology/primary-hemostasis-and-platelets/)
- [The coagulation cascade](https://durgical.com/en/physiology/the-coagulation-cascade/)

## Connections

## Related 4

- [Haemophilia A and B](https://durgical.com/en/general-pathology/haemophilia-a-and-b/)
- [Platelet function disorders](https://durgical.com/en/general-pathology/platelet-function-disorders/)
- [Thrombotic microangiopathies: TTP vs HUS](https://durgical.com/en/general-pathology/thrombotic-microangiopathies-ttp-vs-hus/)
- [Bleeding disorders: overview](https://durgical.com/en/general-pathology/bleeding-disorders-overview/)

## Related conditions 4

- [Von Willebrand disease](https://durgical.com/en/conditions/von-willebrand-disease/)
- [Haemophilia A and B](https://durgical.com/en/conditions/haemophilia-a-and-b/)
- [Abnormal uterine bleeding](https://durgical.com/en/conditions/abnormal-uterine-bleeding/)
- [Epistaxis (nosebleed)](https://durgical.com/en/conditions/epistaxis/)

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: [Von Willebrand disease: MedlinePlus Genetics](https://medlineplus.gov/genetics/condition/von-willebrand-disease/) (MedlinePlus, US National Library of Medicine, 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/general-pathology/von-willebrand-disease/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/von-willebrand-disease/