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Hematology

35+ Years Treating Hemophilia

CSL Behring has over 35 years of experience in the field of hematology. From disorders that span the coagulation cascade to the management of urgent reversal of acquired bleeding, CSL Behring continues to invest in this area and remains committed to continuing our innovations in this field.

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

 

chart of diagnositic process to determine von Willebrand Disease

platelet-dependent VWF activity

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 VWF:RCo and FVIII>50 IU/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 VWF:RCo and FVIII>50 IU/dL for 3-5 days

 

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

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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For U.S. Healthcare Professionals only

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