Von Willebrand disease.

Von Willebrand disease (VWD), the most common inherited bleeding disorder, results from a deficiency of von Willebrand factor (VWF), a protein required for the normal adhesion of platelets to the site of injured endothelium and for preservation of factor VIII in the circulation. The prevalence of VWD has been reported to be as high as 1.3%. Among women with VWD, menorrhagia is the most common symptom, affecting 32% to 100%. Treatments that have been reported to control menorrhagia in these women include combined oral contraceptives, 1-deamino-8-D-arginine vasopressin (DDAVP), tranexamic acid, and the levonorgestrel-releasing intrauterine system. With the exception of nonsteroidal antiinflammatory drugs, any treatments effective in the treatment of menorrhagia, including hysterectomy, may be suitable. Besides menorrhagia, women with VWD appear to be at an increased risk of developing hemorrhagic ovarian cysts and possibly endometriosis. As they grow older, they may be more likely to manifest conditions that present with bleeding such as fibroids, endometrial hyperplasia, and polyps. During pregnancy, they may be at greater risk of miscarriage and bleeding complications, particularly delayed or secondary postpartum hemorrhage. Vaginal or vulvar hematomas, extremely rare in women without bleeding disorders, are not uncommon. Although women with VWD are at risk for the same obstetric and gynecologic problems that affect all women, they appear to be disproportionately affected by conditions that manifest with bleeding.

BACKGROUND

Obstetricians & Gynecologists, Family Physicians.

OBJECTIVE

After completion of this article, the reader should be able to recall that Von Willebrand Disease (VWD) is a common inherited disease, especially in women with menorrhagia; state that prophylaxis therapies against bleeding in pregnant and nonpregnant women are available; and explain that, despite prophylaxis, miscarriage and bleeding complications can still occur.