Does your head spin when you look at a von Willebrand panel? In this episode, Dr. Angela Weyand teaches us about the most typical presentations of VWD, how to interpret those pesky antigen and activity levels, and how DDAVP works for bleeds.
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Von Willebrand factor is a glycoprotein that plays a role in both primary and secondary hemostasis. In primary hemostasis, it binds platelets to exposed collagen on damaged endothelium in order to form the primary platelet plug. In secondary hemostasis, it acts as a chaperone protein for factor VIII to prevent it from being degraded too rapidly.
There can be significant variability in the presentation of VWD, even in patients with similar laboratory findings. Patients frequently report mucocutaneous bleeding including epistaxis, bleeding with dental procedures, easy bruising, or heavy menstrual bleeding. Those with more severe disease may also have muscle/joint bleeding or GI bleeding.
When taking a bleeding history, Dr. Weyand suggests going from head to toe:
The Self-Bleeding Assessment Tool is also an excellent screening tool that can be completed by patients in clinic.
Type 1 (autosomal dominant): This is the most common type of VWD and is found in 70-80% of patients. Type 1 disease is due to a quantitative defect. VWF binds platelets and collagen normally, there just isn’t enough of it. Usually causes mild bleeding.
Type 2 (autosomal dominant): Type 2 disease is due to a qualitative/functional defect.
Type 3 (autosomal recessive): Most severe and <1% of all cases. In these patients, there is a complete deficiency of VWF.
When concerned about a possible bleeding disorder, start by checking:
The von Willebrand panel usually includes three components:
Diagnosis of von Willebrand Disease is made when VWF level (either antigen level or activity level) is <30% regardless of bleeding history, or <50% with a bleeding history.
To determine if a patient has type 1 or type 2 disease, compare the ratio of VWF activity level to VWF antigen level.
Expert Opinion: If referring a patient to hematology, Dr. Weyand suggests NOT checking a von Willebrand panel beforehand because they are best drawn, run, and interpreted in centers with specialized hematology labs (Brown et al 2021).
Most patients with von Willebrand disease will only need episodic treatment for active bleeding and prophylaxis before procedures.
Causes release of endothelially stored VWF and can be used to treat minor bleeding or to increase levels prior to minor procedures. Patients should first undergo a desmopressin trial because there can be variability in response to the medication. Works best for type 1 disease because the factor released in type 2 disease is still functionally impaired. Contraindicated in type 2B disease because it can cause thrombocytopenia and in type 3 disease because of lack of efficacy. Was previously available outpatient in intranasal form, but is now only found as an IV medication (Connell et al 2021).
Used to treat major bleeding or as long-term prophylaxis for patients with severe recurrent bleeding. This includes patients with type 3 disease who are at high risk for hemarthrosis and patients who have significant GI bleeding (Connell et al 2021).
Available in liquid, intranasal, and topical forms to use as adjunctive treatment or prophylactic therapy. Especially helpful for mucocutaneous bleeding (ex. epistaxis, heavy menstrual bleeding, oral bleeding).
Dr. Weyand reminds us of key points to counsel families of patients with VWD about:
Listeners will explain the basic pathophysiology, diagnosis, and management of von Willebrand Disease.
After listening to this episode listeners will…
Dr. Weyand serves as a consultant on medical advisory boards at Genentech, Bayer, Sanofi, and Takeda for which she has received honoraria. The Cribsiders report no relevant financial disclosures.
Mao C, Weyand A, Lee N, Masur S, Chiu C, Berk J. “#64: Von Willebrand Disease.” The Cribsiders Pediatric Podcast. https:/www.thecribsiders.com/ September 21, 2022.
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