Inherited bleeding disorders are under-recognized as causes of bleeding in girls and women. However, recent data have documented that 50% of girls and women who are carriers for hemophilia A or B have factor VIII or IX levels below 50%, putting them in the category of mild hemophilia and causing them to have an increased risk of bleeding, especially during menstruation and at the time of an accident, surgery, or delivery. Moreover, up to 1% of all girls and women may have laboratory evidence of inherited von Willebrand disease (VWD). and may also have heavy bleeding during menstruation, surgery, or labor and delivery. (2)
With this information in mind, MASAC recommends the following:
- Von Willebrand Disease (VWD) and other inherited bleeding disorders should be considered in the differential diagnosis of all girls and women presenting with menorrhagia, and an appropriate workup should be undertaken. Initial testing should include a CBC, PT, PTT, and a TT or fibrinogen. Additional testing specifically for VWD should include factor VIII activity, ristocetin cofactor, Von Willebrand antigen, and Von Willebrand multimers. This workup should be done in consultation with a hematologist who is well versed in the diagnosis of inherited bleeding disorders.
- The above laboratory evaluation should be considered in all girls and women scheduled for endometrial ablation and/or hysterectomy for menorrhagia or dysfunctional uterine bleeding who also have mucous membrane bleeding and/or a family history of bleeding.
- If there is a positive family history of a bleeding disorder, girls and women should have the appropriate factor activity level determined as soon as feasible and definitely prior to any planned surgical procedure regardless of age.
- For bleeding, trauma, and before and after any surgical procedure, or labor and delivery, females with a factor VIII level less than 50% may be treated with recombinant factor VIII or DDAVP (if DDAVP trial has shown her to be responsive).
- If a female with a factor IX level below 50% has a clinically significant bleeding episode, she may be treated with recombinant factor IX.
- For clinical bleeding episodes, females with abnormal Von Willebrand studies may be treated with DDAVP, antifibrinolytics (Amicar or tranexemic acid) or a virally inactivated factor VIII product that contains Von Willebrand factor (Alphanate, Humate P, Koate DVI, Wilate). Alphanate, Humate P, and Wilate are approved by the FDA for treatment of VWD. A recombinant VWF clotting factor product Vonvendi is also available. (See MASAC Document #246, MASAC Recommendations Regarding the Treatment of Von Willebrand Disease, for specific details.)
- Cryoprecipitate and fresh frozen plasma should not be used unless the patient is at risk of life-threatening bleeding and a Factor VIII/Von Willebrand Factor concentrate is not rapidly available. (See MASAC Document #246, MASAC Recommendations Concerning the Treatment of Hemophilia and Related Bleeding Disorders, Revised October 23, 2016).
- Much progress has been made in increasing awareness of girls’ and women’s bleeding disorders by both the general public and clinicians who are primary care providers for girls and women. Nonetheless, the national outreach and education program should be continued. The target audiences should be health care professionals (e.g. pediatricians, hematologists/oncologists, internists, OB/GYN, family practitioners, emergency department personnel and dentists as well as nurse practitioners in these fields), women’s health advocates, and the general public.
- NHF should continue to work with NHLBI and CDC to develop a national research agenda on women’s bleeding disorders.
- Trigg DE, Stergiotou I, Peitsidis P, Kadir RA. A systematic review: The use of desmopressin for treatment and prophylaxis of bleeding disorders in pregnancy. Haemophilia 2012; 18: 25-33.
- James AH, Kouides PA, Abdul-Nadir R, et al Evaluation and Management of Acute Menorrhagia in Women with and without underlying bleeding disorders: consensus from an international expert panel, Eur J Obstet Gynecol Reprod Biol. 2011 Oct; 158(2): 124-34
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