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von Willebrand Disease

Chang L. Lipinski, DO, Jarrett Sell, MD, AAHIVS and Stacey L. Milunic, MD Reviewed 06/2019
 


BASICS

DESCRIPTION

  • von Willebrand disease (vWD) is a bleeding disorder caused by deficiency or a defect of von Willebrand factor (vWF) protein.

  • vWF is critical to the initial stages of blood clotting, a...

DIAGNOSIS

HISTORY

  • Most patients with vWD have a positive family history of a bleeding disorder; however, patients with mild forms of vWD and their families may be unaware of their disease. Those with A...

TREATMENT

GENERAL MEASURES

  • Most patients with type 1 vWD do not require activity restrictions.

  • Patients with type 3 vWD should avoid contact sports.

  • An emergency ID bracelet may be useful.

MEDICATION

First Line

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patients should be seen by a hematologist prior to invasive procedures for determination of perioperative management or advice regarding delivery. 

Patient Monitoring

REFERENCES

1
Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood.  2015;125(13):2029–2037. [View Abstract on OvidMedline]
2
Leebeek FW, Eikenboom JC. Von Willebrand’s di...

ADDITIONAL READING

  • Kessler CM. Diagnosis and treatment of von Willebrand disease: new perspectives and nuances. Haemophilia.  2007;13(Suppl 5):3–14. [View Abstract on OvidMedline]

  • Kumar S, Pruthi ...

CODES

ICD10

D68.0 Von Willebrand’s disease 

ICD9

286.4 Von Willebrand's disease 

SNOMED

  • 128105004 von Willebrand disorder (disorder)

  • 128106003 von Willebrand disease type 1 (disorder)

  • 128107007 von Willebran...

CLINICAL PEARLS

  • vWD varies from a minor to severe bleeding disorder; affects up to 1% of the U.S. population

  • It is important to determine the exact type of vWD (type 1, 2, or 3) to guide treatment.

  • Trea...

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