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Von Willebrand disease
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Von Willebrand disease is a bleeding disorder, meaning a condition in which a person bleeds more than they should, and it comes down to a single protein: von Willebrand factor. There may not be enough of it, which is called a quantitative deficiency, or there may be plenty of it but it works poorly, a qualitative deficiency.
That factor is a glycoprotein involved in hemostasis, the set of mechanisms the body uses to stop bleeding. It is made by endothelial cells, the ones that line the inside of blood vessels, and by megakaryocytes, the giant bone marrow cells that platelets come from. And it does two things: it carries factor VIII, another piece of the clotting system, and it helps platelets stick to the vessel wall when that wall is injured. The glue that sticks platelets to the damaged wall is missing.
That is why the types are separated by what fails: type 1 is a partial shortage of the factor, and type 2 covers defects in its quality. And people who have it may report bleeding with a recognizable pattern: excessive bruising, prolonged bleeding from the mucous membranes, and bleeding that drags on after minor trauma.
In brief
Von Willebrand disease is a bleeding disorder caused by a quantitative or qualitative deficiency of von Willebrand factor.
It is a hemostatic glycoprotein, made by the endothelium and megakaryocytes, that carries factor VIII and supports platelet adhesion after a vascular injury.
The glue that sticks platelets to the damaged wall is missing.
Type 1 is a partial quantitative deficiency and type 2 covers qualitative defects; people who have it may report excessive bruising and prolonged mucosal bleeding or bleeding after minor trauma.
Sources (1)
- National Center for Biotechnology Information, StatPearls PublishingVon Willebrand Disease, StatPearlsPublished
This page explains the topic in general terms; it does not tell anyone what treatment to follow.
Updated