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Therapeutic Areas and Products

Hematology

Von Willebrand Disease

Disease Background

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.

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

Characteristics of Types of VwD

Type Classification Description Bleeding Incidence3
1 Quantitative Partial deficiency of VWF Variable from mild to moderate ~85%
2A Qualitative Defects in multimerization Variable from moderate to severe 10-15%
2B Qualitative Increase in spontaneous platelet binding Variable from moderate to severe  10-15%
2M Qualitative Defects in ligand binding with intact multimers Variable from moderate to severe   10-15%
2N Qualitative Defects in FVIII binding Variable from moderate to severe   10-15%
 3 Quantitative Severe deficiency of VWF Severe  <1%
VWF, von Willebrand factor

 

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

vWD flow chart for diagnostic process

Management Strategies


Treatment Options1,3,4,5

VWD Type Recommended Treatment
1 Desmopressin (if unresponsive to desmopressin use VWF/FVIII concentrates)
2 VWF/FVIII concentrates
3 VWF/FVIII concentrates


VWF concentrate dosing recommendation1

Major surgery/bleeding
Loading dose Maintenance dose Therapeutic goal
40-60 U/kg 20-40 U/kg every 8 to 24 hours Trough WVF:Rco and FVIII>50 IIU/dL for 7-14 days

Minor surgery/bleeding
Loading dose Maintenance dose Therapeutic goal
30-60 U/kg 20-40 U/kg every 12 to 48 hours Trough WVF:Rco and FVIII>50 IIU/dL for 3-5 days

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

Treatment Guidelines

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.

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