Patient case (clinical description): A 23-year-old female has prolonged oral bleeding immediately after a tooth extraction.  Despite several interventions, the bleeding persists for hours and stops only after desmopressin (DDAVP) administration.  The patient has heavy menstrual cycles each month.  She has no other medical problems and takes no medications.  Her mother and grandmother have also had excessive bleeding during menstrual period.  Review of systems is positive for mild bruising on his legs. Laboratory findings reveal a normal platelet count and an abnormal ristocetin cofactor assay, as well as CB <= 0.30 IU/mL and FVIII:C <= 0.40 IU/mL.

Patient's own account: I'm a 23 woman and I went to the ER because of non-stop bleeding after my tooth extraction. I tried to make the bleeding stop but it just kept going for hours and it finally stopped when I took some medication called DDAVP. I usually have heavy periods, just like my mom and grandma had. I don't have any other medical condition and I don't take any other medication. The doctor noticed the bruises on my legs. I had to do some lab tests. I had a normal platelet count but an abnormal ristocetin cofactor assay. I had low CB and FVIII:C levels.

Clinical trial: Extended Half Life Factor (EHF) Products For Heavy Menstrual Bleeding in Hemophilia Carriers

Eligibility criteria:
Inclusion Criteria:

          -  Females of reproductive age who experience monthly menstrual bleedings

          -  Female Hemophilia A or B carrier (heterozygote, lyonized or Turner's Syndrome)

          -  FVIII or FIX activity ≤60% at time of the study

          -  Baseline Pictorial Bleeding Assessment Chart >150 mean at time of recruitment

          -  Negative pregnancy test at time of enrollment

          -  Both female and her male partner have agreed to use an acceptable barrier method of
             birth control (e.g., diaphragm, cervical cap, male condom, female condom, and
             spermicidal foam, sponges, and film) throughout the duration of this study (for
             sexually active participants)

        Exclusion Criteria:

          -  Has not reached menarche

          -  Menopause: natural or induced by surgical/medical treatment

          -  Pregnant or breasfeeding

          -  Female or her male partner refuses to use barrier method of birth control (for
             sexually active)

          -  Current use of any of the following contraceptives (copper IUD, oral combined,
             Progestin-only including but not limited to: Provera, Aygestin, Nexplanon, Implanon,
             Depo Provera, Mirena IUD)

          -  VWF:Ag or VWF:RCo <40%

          -  Diagnosis of a qualitative platelet disorder

          -  Personal history of thrombosis or superficial thrombosis

          -  First degree relative with a history of thrombosis

          -  Personal history of concomitant bleeding or clotting disorder

          -  Cigarette smoker

          -  Willing to avoid taking an anti-fibrinolytic agent (amicar, transexamic acid, lysteda)
             during the trial
Female
No healthy subjects accepted to join the trial.
Subject must be at least 14 Years old.
Subject must be at most 55 Years

Eligibility (NLI label): Entailment