Von Willebrand Disease in Women
Even in the context of gynecological issues, it is sometimes necessary to consider a hematological etiology of difficulties. The recommendations of the American College of Obstetricians and Gynecologists (ACOG) in this context focus on the possible connections with von Willebrand disease.
Introduction
Von Willebrand disease (vWD) is the most common congenital bleeding disorder, with an estimated prevalence among women of around 1%. The overall prevalence is higher in women with significant menstrual bleeding, where various studies estimate it to be between 5 and 25%.
On the other hand, significant menstrual bleeding is one of the relatively common issues in gynecology. Therefore, when performing differential diagnosis, it is also appropriate to consider congenital bleeding disorders.
Directed Inquiry − Screening for Possible Bleeding Issues
One of the important steps is directed inquiry about other possible bleeding manifestations – epistaxis, gingival bleeding, bleeding after tooth extraction or minor injuries, history of significant postoperative or postpartum bleeding, gastrointestinal bleeding, or joint bleeding.
Many adolescent girls escape attention partly due to irregular cycles. It is recommended that every girl with one or more of the following criteria be further examined with regard to the risk of a bleeding disorder:
- menstruation longer than 7 days, very heavy, soaking through a tampon or pad within 2 hours
- history of anemia
- family history of a bleeding disorder
- history of significant bleeding following so-called hemostatic challenges (tooth extraction, surgery, etc.)
Another necessary step is directed inquiry about possible medications the woman is taking that could affect bleeding tendencies.
Positive Screening − Laboratory Tests are Necessary
For women with a positive result from the screening inquiry, laboratory tests are appropriate. An ideal laboratory screen that would completely rule out a possible bleeding disorder does not exist. However, the following initial tests can be indicated:
- complete blood count including platelet count
- prothrombin time (PT) and activated partial thromboplastin time (aPTT)
- thrombin time (TT) and fibrinogen level
All these tests can be indicated and interpreted by a gynecologist or general practitioner. In case of abnormalities or significant suspicion of a hematological disorder despite normal tests, consultation with a hematologist is advisable, who may indicate additional specific tests.
Possible Management of Women with vWD
If vWD is diagnosed, patient care is usually multidisciplinary. Specialized outpatient clinics are beneficial, where a woman can consult both a hematologist and a gynecologist on the same day, allowing an appropriate treatment plan to be established.
There are several treatment options available for women with vWD. Many agree to hormonal therapy, either intrauterine (levonorgestrel-releasing intrauterine system) or systemic hormonal contraception. Non-hormonal therapy includes antifibrinolytics, such as tranexamic acid or aminocaproic acid, which stabilize formed clots. Studies show that antifibrinolytics can reduce menstrual bleeding by 30−55%. They are administered around the expected menstrual period. For more severe forms, replacement therapy with concentrates containing von Willebrand factor/coagulation factor VIII may be indicated.
Conclusion
Von Willebrand disease can be a cause of significant menstrual bleeding. Considering this possibility in the differential diagnosis of abnormal bleeding is crucial, as it can help quickly identify the cause and initiate effective treatment.
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Source: Committee on Adolescent Health Care; Committee on Gynecologic Practice. Committee Opinion No.580: von Willebrand disease in women. Obstet Gynecol 2013; 122 (6): 1368−1373, doi: 10.1097/01.AOG.0000438961.38979.19.
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