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

Abstract

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