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Von Willebrand Disease

Diagnosis and treatment of the most common inherited bleeding disorder.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Von Willebrand Disease?

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting approximately 1% of the population worldwide. It results from quantitative deficiency or qualitative dysfunction of von Willebrand factor (VWF), a glycoprotein essential for platelet adhesion to damaged blood vessels and for stabilizing factor VIII in circulation.

VWD is classified into three main types: Type 1 (partial quantitative deficiency, mild-to-moderate, ~75% of cases), Type 2 (qualitative defect with subtypes 2A, 2B, 2M, 2N), and Type 3 (severe near-total absence of VWF, often with low factor VIII). Most cases are inherited in an autosomal dominant pattern, except Type 3 which is recessive.

Bleeding manifestations are highly variable, ranging from mild mucocutaneous bleeding (epistaxis, easy bruising, heavy menstrual periods) to severe hemorrhage. Many patients are diagnosed in adolescence or adulthood when they encounter surgical or obstetric challenges. Diagnosis requires specialized coagulation testing including VWF antigen, ristocetin cofactor activity, and factor VIII levels.

Symptoms

Frequent or prolonged nosebleeds (epistaxis)
Easy bruising with minor trauma
Heavy or prolonged menstrual periods (menorrhagia)
Prolonged bleeding after dental extractions
Excessive bleeding after surgery or childbirth
Gum bleeding when brushing teeth
Gastrointestinal bleeding (especially in older adults with type 2)
Joint or muscle bleeding (rare, mainly Type 3)

Risk Factors

Family history of bleeding disorder
Female sex (more often diagnosed due to menstruation/childbirth challenges)
Blood type O (associated with lower VWF levels)
Pregnancy (Type 2B can worsen)
Use of antiplatelet or anticoagulant medications (additive bleeding)
Acquired conditions: lymphoproliferative diseases, hypothyroidism, aortic stenosis (acquired VWD)
Stress, illness, or exercise (can transiently mask Type 1 with elevated VWF)
Pediatric onset of unexplained bleeding

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Recurrent nosebleeds lasting more than 10 minutes or requiring nasal packing
  • Heavy menstrual bleeding requiring iron supplementation or transfusions
  • Prolonged bleeding after dental procedures or surgery
  • Family history of bleeding disorders requiring screening
  • Unexplained bruising or petechiae in unusual locations
  • Before any planned surgery or invasive procedure (preoperative evaluation)
  • Postpartum hemorrhage history
  • GI bleeding evaluation when standard workup is negative

Treatment Methods

01
Desmopressin (DDAVP): for Type 1 VWD, releases stored VWF (response varies by type)
02
Von Willebrand factor concentrates (VWF/FVIII): for severe bleeding, surgery, Type 3 patients
03
Tranexamic acid: oral or intravenous antifibrinolytic for mucosal bleeding/menorrhagia
04
Hormonal therapy: combined oral contraceptives or levonorgestrel IUD for menorrhagia
05
Iron supplementation: for chronic anemia from heavy menstrual or GI bleeding
06
Avoidance of antiplatelet drugs (aspirin, NSAIDs) when possible
07
Pre-procedural prophylaxis: VWF concentrate or DDAVP infusion before surgery/dental work
08
Patient education: medical alert identification, hemophilia treatment center follow-up

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.