a Background The levonorgestrel-releasing intrauterine system (LNG-IUS) is used commonly by gynaecologists as a contraceptive and to treat menorrhagia. However, its efficacy has not been examined in women with inherited bleeding disorders. Design A prospective pilot study.Setting A teaching hospital in north London with a designated haemophilia centre.Population Female patients with a known inherited bleeding disorder.Methods Sixteen women with subjective and objective menorrhagia caused by inherited bleeding disorders (13 von Willebrand's Disease, two factor XI deficiency and one Hermansky -Pudlak syndrome), who had previously undergone unsuccessful medical treatment were followed up for nine months after LNG-IUS insertion. Bleeding was measured by pictorial chart and haemoglobin concentration. Results All women reported that their periods were improved, pictorial chart scores were lower and 56% became amenorrhoeic. None reported side effects. Conclusion The LNG-IUS is well tolerated and effective and improves quality of life.

A mail survey of members and fellows of Royal College of Obstetricians and Gynaecologists was carried out to determine current practices of obstetricians and gynaecologists in the United Kingdom in the management of women with inherited bleeding disorders. In total, 3929 questionnaires were sent, 707 returned and analysis was limited to 545 valid questionnaires. In the past 5 years, 91% have managed women with inherited bleeding disorders. The majority (83%) considered inherited bleeding disorders to be under diagnosed in obstetrics and gynaecology. More than 80% considered the prevalence of von Willebrand's disease (VWD) to be <0.2% in the general population and <1% in women with menorrhagia and no gynaecological pathology, although the reported prevalence is 1% and 5-25% respectively. Twelve percent of the respondents would arrange testing for VWD when reviewing an 18-year-old with menorrhagia and no pelvic pathology, while only 2% would do the same for a 35-year-old with the same presentation. Twenty-one percent thought elective caesarean section is indicated in all fetuses known to be at risk of being affected by haemophilia. Eighty-four percent considered vacuum extraction unsafe in these cases, but 76% would consider the use of low forceps. In conclusion, obstetricians and gynaecologists underestimate inherited bleeding disorders as an underlying cause for menorrhagia. Increased awareness and management guidelines are essential in minimizing haemorrhagic complications and improving quality of care of these women.

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A study was conducted to evaluate the value of screening for inherited bleeding disorders in women with primary postpartum haemorrhage (PPH). Over a 2-year period, women identified to have PPH (defined as >500 mL blood loss for spontaneous vaginal delivery, >700 mL for instrumental deliveries and >1000 mL for caesarean sections within 24 h of delivery) were invited to participate in this study testing for a possible underlying bleeding disorder at 3-9 months post-delivery. Women known to have an inherited bleeding disorder were excluded. Of the 5744 deliveries in our unit during the study period, 152 (3%) fulfilled the criteria for primary PPH and 50 women agreed to participate in the study. Of these, 25 (50%) had a spontaneous vaginal delivery, 8 (16%) had an instrumental delivery and 17 (34%) had a caesarean section. Half of the women were multiparous and five (20%) had PPH in their previous pregnancy. Nineteen (38%) and 12 (24%) reported at least one significant personal and family bleeding history, respectively. One (2%) woman was identified to have von Willebrand disease. In conclusion, primary PPH does not appear to be a strong predictor of inherited bleeding disorders. Further studies are required to assess the prevalence of inherited bleeding disorders among these women.

A pilot study was conducted over a 6-month period to evaluate antenatal screening for factor XI (FXI) deficiency amongst Ashkenazi Jewish women booking for their pregnancy in a single obstetric unit. Fifty-four women of Ashkenazi Jewish origin were recruited during their visit for the routine first trimester ultrasound scan. They completed a questionnaire about their personal bleeding symptoms and had blood taken for FXI levels (FXI:C). Seven (13%) women had partial FXI deficiency. Five (9%) were newly diagnosed, and in the remaining two, the diagnosis was known previously. One infant with severe FXI deficiency was identified as a result of maternal testing. This study has shown that FXI deficiency is common amongst women of Ashkenazi Jewish origin and supports its antenatal screening in this population. However, further studies are required to evaluate its cost-effectiveness and the effect on pregnancy outcome.