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's disease. And if you want to follow along with written notes on this

topic, you can follow along at zero definals.com slash von Willa brand or in the hematology section

of the Zero De definals medicine book.

So let's get straight into it.

Bon Wilabrand disease is the most common inherited cause of abnormal bleeding.

And abnormal bleeding is often referred to as hemophilia.

There are many different underlying genetic causes of von Willa Brand disease,

most of which are autosomal dominant, but there are a number that have other inheritance as well.

The causes involve a deficiency or an absence or a malfunctioning of a glycoprotein which is

called von Willibrand factor. So low von Willaubran which is called von WillaBrand Factor.

So low von WillaBrand factor results in von WillaBrand disease.

There are three types which are based on the underlying cause and these range from type 1 to type 3 and type 3 is the most severe.

So how do patients present with von Willaubrand disease?

Well patients present with a history of unusually easy, prolonged or heavy bleeding. So this could be bleeding gums when they brush their teeth, nose bleeds, which we call epistaxis, heavy menstrual periods, which we call menorrhager, or heavy bleeding during surgical operations or childbirth.

A family history of heavy bleeding or von Willebrand disease is also very relevant.

So don't forget to ask about whether members of their family or parents also had problems with

heavy bleeding.

How do we diagnose the condition?

Well, diagnosis is based on history of abnormal bleeding, a family history, bleeding. How do we diagnose the condition? Well, diagnosis is based on history of abnormal

bleeding, a family history, bleeding assessment tools, which are like scoring systems, and laboratory

investigations. But due to all the underlying causes, there's no easy way to just test for

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