[Management of patients with Type 2B von Willebrand's disease during delivery and puerperium]

Von Willebrand's disease (VWD) is the most common congenital haemorrhagic diathesis inherited as an autosomal dominant trait, with a prevalence estimated to be 1 - 2 %. In subtype 2B an abnormally structured von-Willebrand factor (VWF) leads to an increased binding of VWF molecules to normal platelets, which regularly results in thrombocytopenia in pregnancy. Only few systematic observations in patients with type 2B VWD in the perinatal period have been reported in the literature. Six spontaneous deliveries in two sisters with type 2B VWD are reported. The first patient did not show any bleeding complications in five vaginal deliveries without any factor replacement therapy. The second patient showed a massive haemorrhage on the third postpartum day after administration of factor VIII-VWF concentrate replacement therapy (Haemate(R) HS), only on the day of delivery. No neonatal complications were reported. The clinical management of pregnancy, delivery and puerperium in patients with type 2B VWD requires close collaboration of experienced obstetricians, haematologists, anaesthesiologists and paediatricians. During labour and delivery, but especially in puerperium, there is a significantly increased risk for haemorrhage. Vaginal delivery is generally safe, but the incidence of postpartum haemorrhage is 30 %. These bleedings may be extremely severe. The danger of postpartum bleeding complications cannot be predicted with certainty, neither by past history of bleeding episodes or haematological laboratory tests of VWF activity levels. Hence, in all patients factor VIII-VWF concentrate replacement therapy should be initiated already in the first stage of labour. Post partum replacement therapy along with effective uterotonic therapy should be continued at least for seven days. With this treatment bleeding problems may be largely prevented. The decision to perform epidural block in labour and delivery must be assessed depending on individual risk factors.