Advertisement

Von Willebrand Disease: What Women's Health Clinicians Need to Know

Guideline Watch |
December 12, 2013

Von Willebrand Disease: What Women's Health Clinicians Need to Know

  1. Andrew M. Kaunitz, MD

ACOG updates guidance for diagnosing von Willebrand disease, treating heavy menstrual bleeding, and hematologic collaboration in the care of women with this hereditary condition.

  1. Andrew M. Kaunitz, MD

Sponsoring Organization: American College of Obstetricians and Gynecologists (ACOG)

Target Population: Obstetric, gynecologic, and hematologic care providers

Background and Objective

Von Willebrand disease (vWD), an autosomally inherited bleeding disorder in which von Willebrand Factor (a protein important for normal platelet adhesion and proper coagulant factor activity) is deficient, is the most common inherited bleeding disorder among U.S. women. Prevalence of vWD is 1% overall, but can be as high as 24% among women with chronic heavy menstrual bleeding (HMB). In addition to HMB (which is experienced by 74% to 92% of women with vWD), epistaxis, gingival bleeding, and bleeding after dental procedures, from minor cuts and abrasions, and postoperatively are common. These updated guidelines reflect recent clinical advances.

Key Recommendations

Evaluation and Diagnosis

  • In women with HMB whose history strongly suggests a disorder of hemostasis, referral to a hematologist is appropriate.

  • Test results for vWD are affected by pregnancy, use of estrogen–progestin contraceptives, menstrual cycle timing, and laboratory quality.

Treatment

  • Hormonal management of HMB: The levonorgestrel-releasing intrauterine device is effective, as are depot medroxyprogesterone, progestin implants, estrogen–progestin contraceptives, and oral norethindrone acetate.

  • Nonhormonal management of HMB: Tranexamic acid is approved to treat HMB in women without bleeding disorders, but may also be effective for women with vWD. Other therapies include desmopressin acetate (a synthetic vasopressin derivative) and various recombinant clotting factors.

  • Obstetric management: Collaboration with a hematologist is appropriate to plan for delivery and the possibility of postpartum hemorrhage. Regional anesthesia, fetal scalp electrode, and fetal scalp sampling, as well as operative vaginal delivery, should be avoided. Likewise, circumcision should be postponed until the newborn's vWD status is assessed.

  • Adolescent-specific concerns: Heavy bleeding at menarche is often the presenting symptom of vWD. To determine whether a teen with HMB should be evaluated for a possible bleeding disorder, clinicians should inquire about menses lasting >7 days, flooding or gushing sensations, bleeding through pads or tampons in 2 hours, history of anemia, family history of a bleeding disorder, and history of excessive bleeding associated with tooth extraction or surgery. Treatment for HMB in adolescents with vWD is similar to treatment in adult women.

Comment

Von Willebrand disease is an important cause of heavy menstrual bleeding in girls and women. Regardless of a patient's age, women's health clinicians should consider the possibility of a bleeding disorder (including vWD) when evaluating women with HMB. Collaboration with a hematologist optimizes treatment outcomes. The National Heart, Lung and Blood Institute, the National Hemophilia Foundation, and the American Society of Hematology have all produced useful resources for patients and clinicians.

  • Disclosures for Andrew M. Kaunitz, MD at time of publication Consultant / Advisory board Agile; Bayer; Merck Equity Vertex Royalties UpToDate Grant / research support Agile; Bayer; Endoceutics; Medical Diagnostic Laboratories; Noven; Teva Editorial boards Contraception; MedScape; Menopause; OBG Management Leadership positions in professional societies North American Menopause Society (Board of Trustees)

Citation(s):

Reader Comments (3)

Josefina Tan-Domingo MD Physician, Obstetrics/Gynecology, Lewis County General Hospital

I like the shorter version of the articles printed in the Journal Watch vs the articles in the Green Journal. Concise and to the point.

thuy hang nguyen cao

the exam test to dianosis HBM?

Your Comment

(will not be published)

Filtered HTML

  • Allowed HTML tags: <a> <em> <strong> <cite> <blockquote> <code> <ul> <ol> <li> <dl> <dt> <dd>
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Do you have any conflict of interest to disclose?
CAPTCHA
This question is for testing whether you are a human visitor and to prevent automated spam submissions.
Image CAPTCHA
Enter the characters shown in the image.

Vertical Tabs

* Required

Reader comments are intended to encourage lively discussion of clinical topics with your peers in the medical community. We ask that you keep your remarks to a reasonable length, and we reserve the right to withhold publication of remarks that do not meet this standard.

PRIVACY: We will not use your email address, submitted for a comment, for any other purpose nor sell, rent, or share your e-mail address with any third parties. Please see our Privacy Policy.

Advertisement
Advertisement
Advertisement