Von Willebrand disease, the most common inherited bleeding disorder among American women,
is a common cause of heavy menstrual bleeding and other bleeding problems in women and adolescent girls. Von Willebrand disease and other inherited and acquired disorders of coagulation and hemostasis should be considered
in the differential diagnosis of all patients being evaluated for heavy menstrual bleeding, regardless of age.
There are many treatment options available for patients with von Willebrand disease and heavy menstrual bleeding,including hormonal and nonhormonal therapies. A multidisciplinary approach to management, which involves obstetrician–gynecologists and hematologists, results in optimal treatment outcomes.

An autosomally inherited congenital bleeding disorder,
von Willebrand disease involves a qualitative or
quantitative deficiency of von Willebrand factor (vWF),
a protein critical for proper platelet adhesion and protection
against coagulant factor degradation. Dominant
and recessive patterns of inheritance exist. There are
three main types of von Willebrand disease. Type 1 (deficiency
of vWF), the most common, is usually mild; type
2 (abnormal vWF) has several subtypes and is less common;
and type 3 (absence of vWF), which is rare, is the
most severe form.

Presenting Symptoms and Signs
Abnormal uterine bleeding is a commonly reported symptom
among women with a diagnosis of von Willebrand
disease, with 74–92% experiencing heavy menstrual
bleeding . Additional symptoms or signs that may be
present include epistaxis (38–63%), gingival bleeding (26 –35%), bleeding after dental extraction (29–52%),bleeding from minor cuts or abrasions (36%), postoperative
bleeding (20–28%), gastrointestinal bleeding (14%),
and joint bleeding (6–8%).

Evaluation and Diagnosis

Clinical Screening for an Underlying Disorder of Hemostasis in the Adult Patient
With Excessive Menstrual Bleeding
Initial screening for an underlying disorder of hemostasis
in patients with excessive menstrual bleeding should be
structured by the medical history. A positive screening
result* comprises the following circumstances:
• Heavy menstrual bleeding since menarche
• One of the following conditions:
—Postpartum hemorrhage
—Surgery-related bleeding
—Bleeding associated with dental work
• Two or more of the following conditions:
—Epistaxis, one to two times per month
—Frequent gum bleeding
—Family history of bleeding symptoms

Positive initial screen result by history and physical examination
Initial hemostasis tests
• CBC and platelet count
• PT and PTT
• Fibrinogen or TT (optional)
If bleeding history is strong, consider performing initial von
Willebrand disease assays in conjunction with hematologist

Other cause identified, eg, decreased platelets,
isolated abnormal PT, low fibrinogen, and abnormal TT

Referral to hematologist for other appropriate evaluation

Isolated prolonged PTT that corrects on 1:1 mixing
study, or no abnormalities

Referral to hematologist for initial
von Willebrand disease assays
• vWF:Ag
• vWF:RCo


Hormonal Treatments

Nonhormonal Treatments

Gynecologic Considerations
The association of von Willebrand disease with other
gynecologic problems––including ovarian cysts, endometriosis,
and leiomyomas––is uncertain . Heavy
menstrual bleeding or hemorrhagic ovarian cysts may
be managed with combined hormonal contraceptives,
which can address both the bleeding and the development
of hemorrhagic cysts . For the acute presentation
of a ruptured ovarian cyst, patients with von
Willebrand disease may require surgical intervention for

Obstetric Considerations ^
Obstetric concerns regarding patients with bleeding disorders
include spontaneous abortion, mode of delivery,
epidural management, operative delivery techniques, and
postpartum hemorrhage. Patients with an underlying
bleeding disorder are at a high risk of epidural or spinal
hematoma . Many experts advocate that women
with von Willebrand disease may have a vaginal delivery
safely, with cesarean delivery reserved for standard indications.

Recommended Screening Tool for Adolescent Patients Who Report Heavy Menstrual Bleeding

If a patient meets one or more of the following criteria,
it indicates a positive screen result and warrants further
1. Menses greater than 7 days and “flooding” or “gushing”
sensation or bleeding through pad or tampon in
2 hours
2. History of anemia
3. Family history of bleeding disorder
4. History of bleeding disorder after hemostatic challenge
(ie, tooth extraction, surgery, delivery)

Von Willebrand disease is a common cause of heavy
menstrual bleeding and other bleeding problems in
women and adolescent girls. Obstetrician–gynecologists
should include von Willebrand disease and other bleeding
disorders in the differential diagnosis when evaluating
patients with heavy menstrual bleeding, regardless
of age. Once a diagnosis is established, collaboration with
a hematologist is recommended for the long-term care of
patients with bleeding disorders, such as von Willebrand
disease. Many resources exist for patients and health care
providers through the National Heart, Lung, and Blood
Institute; National Hemophilia Foundation; and the
American Society of Hematology.