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Von Willebrand Disease (VWD)
Part 1: NHLBI Diagnosis Guidelines

Introduction & Clinical Assessment Recommendations



VWD: Prevalence, Inheritance, Symptoms

Slide 7

May 2011

VWD is the most common inherited bleeding disorder worldwide, with estimates of prevalence up to 1% (or 1 in 100 persons); but this depends on definitions, so the clinical prevalence is probably somewhat lower, such as 1 in 500 or 1 in 1000 (0.1%). About 75% of clinically affected persons have Type 1 VWD of variable severity, whereas almost all the remainder have Type 2 variants, and Type 3 VWD is rare (approximately 1 in 1 million).

VWD inheritance is generally autosomal dominant, except for autosomal recessive inheritance for Type 3 VWD. Bleeding symptoms in VWD are usually mild to moderate in severity (ie, the bleeding does not require physician visits or blood transfusions), but can sometimes be more serious (or life-threatening), especially in more severe VWD such as with Type 3 or some Type 2 VWD variants. Bleeding in VWD mainly involves mucous membranes or skin sites (mucocutaneous bleeding) such as: nosebleeds (epistaxis), excessive skin bruising (ecchymosis), menorrhagia in women, or gastrointestinal (GI) bleeding. Provoked bleeding, such as with or following surgery or other invasive procedures, or with trauma or childbirth (obstetrical bleeding), can be troublesome, partly depending on the underlying VWD subtype and severity.

As a group, women with VWD are more clinically affected than men with VWD, because of the hemostatic challenges of menstruation and childbirth, as well as ovulation (eg, the risk of hemorrhagic ovarian cysts is increased in women with VWD). Hemarthroses (intra-articular joint bleeds) and tissue hematomas are uncommon in VWD, except for Type 3 (severe) VWD, in contrast to their frequency in Hemophilias A or B (hereditary X chromosome-linked deficiencies of coagulation factors VIII or IX).

VWD: Prevalence, Inheritance, Symptoms

 


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