Women and inherited bleeding disorders: menstrual issues.

Von Willebrand, who described a bleeder family from Aland in 1926 in whom the index patient died at the fourth menses, stated, "the trait seems to be seen among women." Menorrhagia is defined objectively as a menstrual blood loss of at least 80 mL. However, even though 5% of women aged 30 to 49 years consult their general practitioner and 12% of gynecologic referrals are for menorrhagia, the diagnosis is difficult. A pictorial bleeding assessment chart (PBAC) has a specificity and sensitivity of more than 80%, with a score of > or = 100 being equivalent to more than 80 mL of blood loss. Using this chart to screen 150 women with menorrhagia who attended a gynecology clinic, an inherited bleeding disorder was diagnosed in 17%. Menorrhagia with onset at the menarche was predictive of an inherited bleeding disorder in 65% of von Willebrand's disease (vWD) and 67% of factor XI (FXI)-deficient patients. A retrospective survey in patients with inherited bleeding disorders using the PBAC showed menorrhagia in 74% of patients with vWD, 57% of carriers of hemophilia A or B, and 59% of FXI-deficient patients, compared with 29% of control individuals. Menorrhagia was the most common symptom in 60% of patients with FVII deficiency studied. Menorrhagia in women with bleeding disorders can be controlled with tranexamic acid with good effect. More recently, a desmopressin acetate (DDAVP) spray has been shown to achieve good FVIII and von Willebrand factor (vWF) levels and is efficacious for women with these deficiencies. The oral contraceptive pill may be useful. Since bleeding disorders are found in a substantial number of women with menorrhagia, it is important that such patients are investigated for these disorders before invasive procedures are done; hysteroscopy and hysterectomy in these patients are associated with a high rate of postoperative bleeding.