PREGNANCY IN WOMEN WITH VON-WILLEBRANDS-DISEASE OR FACTOR-XI DEFICIENCY

Objective To assess the obstetric outcome in women with von Willebrand 's disease or factor XI deficiency. Setting Haemophilia Centre and Hae mostasis Unit, The Royal Free Hospital. Population Women with von Will ebrand's disease (n = 31) and with factor XI deficiency (n = 11) regis tered at the Royal Free Hospital Haemophilia Centre who had had a preg nancy within the previous 17 years (1980-1996), including 84 in women with von Willebrand's disease and 28 in women with factor XI deficienc y.Methods Women were interviewed and details of the obstetric history were obtained. The records of the Haemophilia Centre and the women's m aternity records were also reviewed. Results Threatened miscarriage oc curred in 33% and 14% of pregnancies with von Willebrand's disease and factor XI deficiency, respectively. Excluding recurrent miscarriages, 14/68 (21%) of pregnancies with von Willebrand's disease and one preg nancy with factor XI deficiency miscarried spontaneously. There was an increased incidence of primary and secondary post-abortal bleeding co mplications. Factor VIII and von Willebrand factor antigen and activit y levels increased significantly in pregnancy in all women apart from those with severe von Willebrand's disease. Factor XI, however, did no t show any significant change. No neonatal haemorrhagic complications in association with the birth process were reported, although ventouse and difficult forceps deliveries were avoided. Extensive perineal bru ising and haematoma was reported in three women with von Willebrand's disease; two of these were associated with forceps delivery. The incid ence of primary postpartum haemorrhage was 18.5% in von Willebrand's d isease and 16% in factor XI deficiency. Blood transfusion was required in six cases of von Willebrand's disease and two cases of factor XI d eficiency. Ten of fourteen instances of primary postpartum haemorrhage occurred when maternal factor levels were < 50 IU/dL with no prophyla ctic treatment for labour. The incidence of secondary postpartum haemo rrhage was 20% in von Willebrand's disease and 24% in factor XI defici ency. None of the women who had prophylactic treatment during labour o r the puerperium suffered any significant bleeding complications. Ther e were three neonatal bleeding complications. Conclusion Pregnancy, la bour and the puerperium are associated with significant bleeding probl ems in women with von Willebrand's disease or factor XI deficiency, bu t these are largely preventable. Specialist obstetric care in close li aison with the haemophilia centre is essential to minimise maternal an d neonatal complications.