MASAC RECOMMENDATIONS REGARDING WOMEN WITH INHERITED BLEEDING DISORDERS
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The following recommendations were approved by the Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation on October 14, 2006, and adopted by the NHF Board of Directors on October 15, 2006.
Inherited bleeding disorders are underrecognized as causes of bleeding in women. However, recent data have documented that 50% of 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 or surgery. Moreover, 1%-2% of the general population may have laboratory evidence of von Willebrand disease. Thus 1%-2% of all women may have inherited this bleeding tendency and may also have heavy bleeding during menstruation or surgery.
With this information in mind, MASAC recommends the following:
1. von Willebrand disease (vWD) and other inherited bleeding disorders should be considered in the differential diagnosis of all females 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 factor VIII-related multimers. This workup should be done in consultation with a hematologist who is well versed in the diagnosis of inherited bleeding disorders.
2. The above laboratory evaluation should be considered in all females 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.
3. If there is a positive family history of hemophilia, females should have a factor VIII or factor IX activity level determined as soon as feasible and definitely prior to any planned surgical procedure regardless of age.
4. For bleeding, trauma, and before and after any surgical procedure, females with a low factor VIII level (less than 50%) may be treated with recombinant factor VIII or DDAVP (if DDAVP challenge has shown her to be responsive). Females with a low factor IX level (below 50%) should be treated with recombinant factor IX. Females with abnormal von Willebrand studies should be treated with DDAVP or a virally inactivated factor VIII product that contains von Willebrand factor (AlphaNate, Humate P, Koate HP). Humate P is currently the only product approved by the FDA for treatment of VWD. (See MASAC Document #173, 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. (See MASAC Document #177, MASAC Recommendations Concerning the Treatment of Hemophilia and Related Bleeding Disorders, Revised October 2006).
B. Outreach, Education, and Research
1. Because women’s bleeding disorders are underrecognized by both the general public and clinicians who are primary care providers for girls and women, a national outreach and education program should be mounted. The target audiences should be health care professionals (e.g. pediatricians, hematologists/oncologists, internists, OB/GYN, family practitioners, and dentists as well as nurse practitioners in these fields), women’s health advocates, and the general public.
2. NHF should continue to work with NHLBI and CDC to develop a national research agenda on women’s bleeding disorders.