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

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.