Gynaecological problems and choices for contraception in women with inherited bleeding disorders.

©FSRH J Fam Plann Reprod Health Care 2008: 34(2) Introduction Inherited bleeding disorders (IBDs) in women are more common than previously suspected. Women with IBDs are at risk of heavy bleeding from haemostatic challenges such as menstruation and childbirth. These common haemostatic challenges can cause symptoms even in mildly affected women, thus increasing the clinical prevalence of IBDs in women compared to men.1 von Willebrand disease (VWD) is the most common IBD. This has a reported prevalence in the general population of 0.8–1.3%.2,3 It is an autosomal disorder resulting from defects in von Willebrand factor (VWF), a protein necessary for haemostasis and an integral part of platelet adhesion and thrombus formation. VWF also acts as a carrier for factor VIII (an essential clotting factor) in the circulation.4 VWD can be a result of a quantitative (Types 1 and 3) or qualitative (Type 2) defect. Type I VWD accounts for 70% of all cases with considerable variation in severity. The majority of cases are mild and patients may remain asymptomatic until times of haemostatic challenges such as major trauma or surgery.5 Type 2 VWD accounts for 15–20% of cases and is characterised by abnormal structures of VWF that affect interaction between platelets, vessel walls and VWF. Type 3 VWD is the least common but most severe form of the disease. It is an autosomalrecessive disorder characterised by a severe deficiency of VWF and factor VIII. Haemophilia A and B are X-linked IBDs characterised by deficiencies of clotting factor VIII or IX, respectively. Men inherit the condition whilst women are affected as carriers. The incidence of haemophilia A and B in the general population is 1 in 101000 and 1 in 601000, respectively,6 but the carrier rates in women are unknown. A wide range of factor VIII or IX levels have been reported among carriers of haemophilia, from the upper limit of normal through to very low levels similar to affected males caused by random X-chromosome inactivation (lyonisation).7 Thus carriers may have an increased bleeding tendency and experience significant bleeding symptoms. In a study by Plug et al. carriers were found to experience more spontaneous and provoked haemorrhage compared to non-carriers in families with haemophilia.8 Gynaecological problems and choices for contraception in women with inherited bleeding disorders