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von Willebrand Disease (vWD)

Chronic Lymphocytic Leukemia (CLL) is a chronic lymphoproliferative malignancy characterized by the accumulation of mature but functionally incompetent B lymphocytes in the blood, bone marrow, lymph nodes, and spleen. It is the most common leukemia in adults in Western countries.

Also called

von Willebrand Syndrome (vWD)

ICD-10

D68.0

Specialty

Hematology

Onset

Chronic

Reviewed

August 2026

On This Page

Overview

von Willebrand Disease (vWD) results from reduced amount or impaired function of von Willebrand factor. von Willebrand factor is important for platelet adhesion and carries and stabilizes factor VIII in the circulation. Patients typically develop recurrent mucosal bleeding, easy bruising, and prolonged menstrual bleeding.

Etiology & Risk Factors

-Etiology

von Willebrand Disease (vWD) is usually inherited and results from:

  • Reduced von Willebrand factor production

  • Abnormal von Willebrand factor function

  • Complete or near-complete absence of von Willebrand factor in severe disease

-Types

Type 1

  • Partial quantitative deficiency

  • Most common

  • Usually mild

-Type 2

Qualitative defect in von Willebrand factor.

Subtypes:

  • Type 2A

  • Type 2B

  • Type 2M

  • Type 2N

-Type 3

  • Severe quantitative deficiency

  • Very low or absent von Willebrand factor

  • Severe bleeding

-Acquired von Willebrand Syndrome

Can occur secondary to:

  • Cardiovascular disorders

  • Lymphoproliferative disorders

  • Myeloproliferative disorders

  • Autoimmune disease

  • Some malignancies

Pathophysiology

Defective or deficient von Willebrand factor → impaired platelet adhesion to damaged vascular endothelium → impaired primary hemostasis → mucocutaneous bleeding → reduced stabilization of factor VIII → reduced factor VIII activity in some patients → prolonged bleeding tendency

Clinical Presentation

Symptoms

  • Easy bruising

  • Recurrent epistaxis

  • Gum bleeding

  • Heavy menstrual bleeding

  • Prolonged bleeding after dental procedures

  • Prolonged bleeding after surgery

  • Prolonged bleeding from minor wounds

  • Postpartum bleeding

Severe Disease

May cause:

  • Gastrointestinal bleeding

  • Hemarthrosis

  • Deep tissue bleeding

These are more typical of severe disease, particularly Type 3.

Signs

  • Petechiae may occur

  • Easy bruising

  • Mucosal bleeding

  • Pallor if chronic blood loss has caused anemia

History Taking

-General Examination

Look for:

  • Pallor

  • Signs of anemia

  • Bruising

-Skin Examination

Assess for:

  • Ecchymoses

  • Petechiae

-Oral Examination

Look for:

  • Gum bleeding

  • Mucosal bleeding

-Nasal Examination

Look for:

    • Evidence of recurrent epistaxis

Physical Examination

-General Examination

Look for:

  • Pallor

  • Fever

  • Weight loss

  • Petechiae

  • Ecchymoses

-Lymphatic Examination

Assess for:

  • Cervical lymphadenopathy

  • Axillary lymphadenopathy

  • Inguinal lymphadenopathy

-Abdominal Examination

Assess for:

  • Splenomegaly

  • Hepatomegaly

Investigations

-Complete Blood Count

Usually:

  • Platelet count: normal

  • Hemoglobin may be reduced if chronic bleeding has caused iron deficiency anemia

-PT

Usually:

  • Normal

-aPTT

  • Usually normal

  • May be prolonged when factor VIII is significantly reduced

-von Willebrand Factor Antigen

Measures the amount of von Willebrand factor in plasma.

May be:

  • Reduced in quantitative deficiency

  • Normal in some qualitative disorders

-von Willebrand Factor Activity

Assesses the ability of von Willebrand factor to function properly.

May demonstrate:

  • Reduced platelet-dependent activity

  • Disproportionate reduction in activity compared with antigen in some Type 2 disorders

-Factor VIII Activity

May be:

  • Normal

  • Reduced, particularly in severe disease or Type 2N disease

-Platelet Function Testing

May be used when the diagnosis or subtype remains unclear.

Multimer Analysis

Can help distinguish different qualitative types of von Willebrand Disease (vWD), particularly Type 2 subtypes.

-Blood Group

Blood group can influence baseline von Willebrand factor levels, with lower levels commonly seen in individuals with blood group O.

Diagnosis

Diagnosis is based on:

  • Compatible personal bleeding history
  • Family history
  • von Willebrand factor antigen
  • von Willebrand factor activity
  • Factor VIII activity
  • Additional specialized testing when required
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Management

1. Desmopressin (DDAVP)

Used in appropriate patients, particularly many patients with Type 1 von Willebrand Disease (vWD).

It increases the release of stored:

  • von Willebrand factor

  • Factor VIII

A DDAVP trial is often used to determine responsiveness before relying on it for procedures.

2. von Willebrand Factor Replacement

Used when:

  • Desmopressin is ineffective

  • Desmopressin is contraindicated

  • Severe disease is present

  • Major surgery or significant bleeding occurs

Preparations containing von Willebrand factor ± factor VIII may be used.

3. Antifibrinolytic Therapy

Useful for mucosal bleeding and dental procedures.

Example:

  • Tranexamic acid

4. Heavy Menstrual Bleeding

Management may include:

  • Tranexamic acid

  • Hormonal therapy

  • Levonorgestrel-releasing intrauterine system

  • Desmopressin in selected patients

5. Iron Replacement

Treat iron deficiency caused by chronic blood loss.

Complications

  • Iron deficiency anemia
  • Severe bleeding
  • Postoperative hemorrhage
  • Postpartum hemorrhage
  • Gastrointestinal bleeding
  • Rare hemarthrosis in severe disease
  • Reduced quality of life from recurrent bleeding

Prognosis

  • Most patients with von Willebrand Disease (vWD) have a mild to moderate bleeding tendency and can lead normal lives with appropriate management. Severe disease requires careful planning before surgery, dental procedures, pregnancy, and childbirth.

Key Points / Clinical Pearls

  • von Willebrand Disease (vWD) is the most common inherited bleeding disorder.
  • It is caused by deficiency or dysfunction of von Willebrand factor.
  • von Willebrand factor is important for platelet adhesion and factor VIII stabilization.
  • Typical bleeding is mucocutaneous.
  • Common symptoms include epistaxis, easy bruising, and heavy menstrual bleeding.
  • Platelet count is usually normal.
  • PT is usually normal.
  • aPTT may be normal or prolonged.
  • Diagnosis requires von Willebrand factor antigen + activity + factor VIII assessment.
  • Desmopressin is useful in many patients with Type 1 disease.
  • von Willebrand factor replacement is used for severe bleeding and major procedures when needed.
  • Tranexamic acid is useful for mucosal bleeding.