Von Willebrand Disease

Von Willebrand Disease (VWD) is a genetic disease that interferes with clotting; it is inherited equally by boys and girls.  About 1 in 100 people have it to some degree, but nothing ever happens to the vast majority.  Only about 1 in 10,000 people have significant disease.

About 75% of pts have Type-1, which can range from mild to severe.  The other 25% of pts have up to 6 or more other variations, requiring more complicated testing.

Physical examination is usually completely normal.  We think of VWD if a patient has: 1) easy bruising; 2) easy bleeding from skin injury; or 3) heavier-than-normal bleeding from mucus membranes, like nosebleeds, dental work, or menstrual periods.  But each of these categories requires some explanation. 

For example, we don’t pay attention to small bruises on arms or legs, especially among women, because this is very common.  We also don’t worry if a patient has taken aspirin within a week, or takes NSAID medication every day, because that can easily cause bruising.  Nosebleeds lasting under 10 minutes aren’t significant.  Menstrual bleeding is significant if it interferes with work (in or out of the house), exercise, or social activities.

There are very detailed questionnaires to help determine whether bleeding is significant; here’s one of them, that’s complex enough that a trained Hematologist (blood specialist) should help administer it: (https://c.ymcdn.com/sites/www.isth.org/resource/resmgr/ssc/isth-ssc_bleeding_assessment.pdf)

There are some basic screening blood tests, with different methods for performing them, and different ways of interpreting them.  Most clinicians are unfamiliar with them.  If a patient seems to have unusual or excessive bleeding, we first order basic tests for other diseases:

If these 3 tests are normal, & we wondered about VBD, we’d refer to a Hematologist.  If any of the basic screening tests are abnormal, more detailed testing is necessary, so we might as well let a specialist handle it all.  We’d certainly refer if our patient had family members with WBD.

Treatment depends on the Type of VWD, and in what situations it would be required.  Another reason that Hematology referral is recommended.

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