von Willebrand Disease| CSL Behring Medical Affairs

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Disease Background

vwd overview 2 Von Willebrand Disease (VWD) is an autosomal inherited bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (VWF) and is the most common bleeding disorder, found in up to 1% of the US population.

VWF, along with factor VIII (FVIII), is a key molecule in platelet aggregation and adhesion and is critical to prevent bleeding via blood clotting.

─VWF and FVIII circulate as a tightly bound complex

─VWF increases the half-life and stability of FVIII in circulation

Patients with reduced VWF have reduced FVIII

Typical symptoms of VWD include easy bruising, epistaxis, gingival bleeding, surgical bleeding, and heavy menstrual bleeding.

Characteristics of Types of VWD

1. 3.Sharma R, Flood VH. Advances in the diagnosis and treatment of Von Willebrand Disease. Hematology Am Soc Hematol Educ Program. 2017(1):379–384. 4.Ng C, Motto DG, Di Paola J. Diagnostic Approach to VWD. Blood 2015; 125(13):2029–2037. 5. Federici A. Management of inherited Von Willebrand Disease in 2006.. Semin Thrombosis Hemostasis. 2006; 32:616–620.

Diagnostic Process

The National Institute of Health recommends that assessments for diagnosis of VWD occur in two main stages:

1. Evaluation of clinical history

Family history and personal bleeding history (spontaneity and severity, sites of bleeding, duration of bleeding, type of injury associated with bleeding, ease with which bleeding can be stopped)

2. Laboratory evaluations

chart of diagnositic process to determine von Willebrand Disease

^aplatelet-dependent VWF activity

VWD Treatment Options 2

VWD dosing recommendations 2

* Antifibinolytic agents in combination with topical agents, or with desmopressin and VWF/FVIII concentrates, can also be used¹

ASH ISTH NHF WFH 2021 Guidelines on the Diagnosis and Management of von Willebrand Disease

References:

1.Nichols WL, Hultin MB, James AH, et al. Von Willebrand Disease. Haemophilia 2008;14(2):171–232.2.Data and statistics on von Willebrand disease. Available from: https://www.cdc.gov/ncbddd/vwd/data.html 3.Sharma R, Flood VH. Advances in the diagnosis and treatment of Von Willebrand Disease. Hematology Am Soc Hematol Educ Program. 2017(1):379–384. 4.Ng C, Motto DG, Di Paola J. Diagnostic Approach to VWD. Blood 2015; 125(13):2029–2037. 5. Federici A. Management of inherited Von Willebrand Disease in 2006.. Semin Thrombosis Hemostasis. 2006; 32:616–620.