Women and von Willebrand disease: controversies in diagnosis and management.

von Willebrand disease (vWD) is the commonest bleeding disorder in women. More than 70% of women with vWD suffer from menorrhagia and half of them suffer from dysmenorrhea. There is also the risk of hemorrhage with ovulation and mid-cycle pain. These have a significant effect on all aspects of quality of life. vWD also can be the underlying cause of menorrhagia in a small but significant proportion of women. There are still several unanswered issues in the diagnosis and management of menorrhagia in these women. There is no consensus whether testing for vWD should be part of the routine investigations in menorrhagia. Diagnosis of vWD is difficult. There are intraindividual variations in von Willebrand factor and factor VIII levels influenced by age, race, and blood group. This is further complicated in women because of the fluctuation of these factor levels during the menstrual cycle and possibly with hormonal therapy. The diagnosis of menorrhagia is also difficult due to the lack of a simple objective tool for the assessment of menstrual blood loss. In vWD, the treatment of menorrhagia is usually medical, but there is lack of prospective data on the efficacy of commonly used medical therapies in these women. The levonorgestrel intrauterine system, Mirena, is effective and should be considered prior to surgical management. Surgical interventions may be required in patients unresponsive to medical treatments. These procedures can be complicated by hemorrhage in these women. A multidisciplinary approach in the management of these women is essential in ensuring an optimal outcome. Multicenter clinical trials are required to answer the controversial issues in the management of women with vWD.