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Bleeding disorders can create problems with reproductions.
Management of reproductive issues in women and girls with bleeding disorders requires knowledge of both reproductive physiology and hematology. Ob/gyns may not be familiar with a particular blood disorder or the latest therapy for its management, whereas hematologists most likely are not prepared to manage impact on aspects of reproduction including menstruation, ovulation, gestation, and delivery.
This article reviews some key concerns for ob/gyns faced with caring for women with common nonmalignant blood disorders.
Prevalence and consequences
Menorrhagia is common in women with bleeding disorders and prevalence ranges from 10% to 100%, depending on the specific bleeding disorder.1 Bleeding disorders also are more prevalent in women with menorrhagia: Studies indicate that 5% to 32% of women with menorrhagia have bleeding disorders. Although menorrhagia is the most common reproductive-tract manifestation of a bleeding disorder, it is not the only sign. Women with bleeding disorders also appear to have an increased risk of developing hemorrhagic ovarian cysts and possibly endometriosis.
Women suspected of having a bleeding disorder (or who are hemophilia carriers) should undergo diagnostic testing before becoming pregnant to ensure appropriate preconception counseling and pregnancy management. During pregnancy, women with bleeding disorders may have an increased risk of bleeding complications. At delivery, affected women are more likely to experience postpartum hemorrhage, particularly delayed or secondary postpartum hemorrhage.
As women with bleeding disorders age, they may be more likely to experience conditions that present with bleeding, such as fibroids, endometrial hyperplasia, and polyps.1 They are also more likely to undergo hysterectomy and to have the procedure at a younger age. In a survey of women with VWD enrolled in US hemophilia treatment centers, 25% reported having had a hysterectomy, compared with 9% of matched controls.2 Women with bleeding disorders are at risk of the same obstetric and gynecologic problems that affect all women, but they appear to be disproportionately affected by conditions that manifest with bleeding (see "Resources for physicians").1
von Willebrand disease
VWD is the most common inherited bleeding disorder.1 It is caused by deficiency in or dysfunction or absence of von Willebrand factor, which is necessary for platelet adhesion at sites of vascular injury and for protection of clotting factor VIII from proteolysis in the circulation.3 VWD manifests with mucocutaneous bleeding, including uterine bleeding. The prevalence of VWD in the general population ranges from 0.6% to 1.3%, depending upon the number of individuals with bleeding symptoms, family history, and laboratory abnormalities. VWD affects both men and women, but its implications for women are more serious than for men because they experience menstruation, pregnancy, and childbirth.4 Among women with menorrhagia, prevalence of VWD is reportedly 5% to 20%, and it may be as high as 36% in adolescents with menorrhagia.4 VWD is of en overlooked in women and girls because of lack of awareness among providers and patients. Bleeding symptoms of en manifest in the reproductive tract, so clinicians may diagnose and treat the condition as a gynecologic problem without appreciating the contribution of the underlying bleeding disorder.
In 2004, the National Heart, Lung, and Blood Institute of the National Institutes of Health convened a panel of experts to develop evidence-based practice guidelines for diagnosis, evaluation, and management of VWD by primary care providers and specialists. The guidelines were published in 2008 and excerpted for ob/gyns in Obstetrics and Gynecology in 2009.5 Other consensus guidelines have been published by various government agencies and hemophilia organizations, as summarized in Trombosis Research in 2009.6 Te American College of Obstetricians and Gynecologists (ACOG) issued a Committee Opinion on VWD in women in 2009.7