Obstetric and gynaecological aspects of von Willebrand disease.

The impact of von Willebrand disease in females is pronounced in terms of menorrhagia and postpartum haemorrhage. There is a very high proportion of von Willebrand disease patients with menorrhagia and associated anaemia, impairment of quality of life, including loss of time from work or school, and a high rate of the use of hysterectomy for ultimate control of the bleeding. The 'early' detection of von Willebrand disease in females may avert these complications. Consequently, there have recently been ongoing international efforts to determine the prevalence of von Willebrand disease in females presenting with menorrhagia, providing a prevalence of 7-20% combined from three studies including a total of 300 patients. Issues remain regarding the optimal dose/schedule of intranasal or subcutaneous desmopressin use for menorrhagia and the relative efficacy of anti-fibrinolytic agents. The proper role of oral contraceptives deserves further study in von Willebrand disease patients with menorrhagia as recent studies have paradoxically demonstrated a lower response rate in type 1 than type 2 or 3 von Willebrand disease. Despite the well-known adage of the 'gestational palliation' of von Willebrand disease, there is also a high proportion of postpartum haemorrhage in type 1 patients, especially after the 24 hour post-delivery period. This may occur despite a normalization of the factor VIIIc level in the third trimester, particularly in type 2 and 3 patients. The care-giver must be aware that haemorrhage can occur up to 5 weeks postpartum. In sum, studies over the past decade have documented a substantial impact of menses and childbirth on von Willebrand disease patients. These results should serve as a basis for interventional studies to reduce the morbidity of menstruation and childbirth.