MASAC Recommendations #185: Regarding Women with Inherited Bleeding Disorders
The following recommendations were approved by the Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation on November 15, 2008, and adopted by the NHF Board of Directors on November 16, 2008.
Inherited bleeding disorders are under-recognized 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:
A. Management
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 von Willebrand 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). If a female with a low 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 or a virally inactivated factor VIII product that contains von Willebrand factor (Alphanate, Humate P, Koate HP). Alphanate and Humate P are approved by the FDA for treatment of VWD. (See MASAC Document #186, 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 #187, MASAC Recommendations Concerning the Treatment of Hemophilia and Related Bleeding Disorders, Revised November 2008).
B. Outreach, Education, and Research
1. Much progress has been made in increasing awareness of 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, 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.
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This material is provided for your general information only. NHF does not give medical advice or engage in the practice of medicine. NHF under no circumstances recommends particular treatment for specific individuals and in all cases recommends that you consult your physician or local treatment center before pursuing any course of treatment. |