Hello and welcome to the Zero to Finals podcast. My name is Tom and in this episode I'm going to be

talking to you about Von Willa Brand Disease. And you can find written notes on this topic at

zero to finals.com slash von Willa Brand disease or in the hematology section of the second edition of the zero to

finals medicine book. So let's get straight into it. Von Willabrand's disease is the most common

inherited cause of abnormal or prolonged bleeding. There are many underlying genetic causes, most of which are autosomal dominant.

There is a rarer, acquired version of von Willabrand disease, which is usually secondary

to an underlying disease, for example, leukemia.

In von Willauban disease, there is a deficiency, absence or malfunctioning of a glycoprotein

called von Willa Brand Factor. Von Willa Brand Factor is important in platelet adhesion and aggregation

or platelets sticking together inside damaged vessels.

A problem with von Willa brand factor means that platelets don't stick or clump together as well as they should do.

There are three types of von Willa Brand disease.

Type 1 involves a partial deficiency of von Willa brand factor and is the most common and the mildest type.

Type 2 involves the reduced production of von Willa brand factor and type 3 involves a complete

deficiency of von Willa brand factor Factor, and this is the

most rare and the most severe type.

Let's talk about the presentation.

Patients present with a history of unusually easy, prolonged, or heavy bleeding.

For example, bleeding gums after brushing their teeth,

nosebleeds or epistuxus, easy bruising, heavy menstrual periods or menorrhager,

and heavy bleeding during and after surgical operations. A family history of heavy bleeding, for example, Menorrhager or von Willa brand disease, is relevant,

as it's a genetic condition most of the time.

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