Postpartum bleeding and von Willebrand's disease

We calculated the decreases in hemoglobin levels between the day of delivery and postpartum day 3 in all 3565 deliveries of the year 1997. Fourteen patients had a drop in hemoglobin level of more than 8% and were included in the study. Ten women without excessive bleeding served as a control group. In all women tested, plasma von willebrand factor antigen (vWF:Ag) correlated well with factor VIII:C activity, and both were within normal limits, so that we were able to exclude the presence of type I von willebrand′s disease (vWD I). Two women in the excessive bleeding group were found to have an isolated decrease in coagulation factor activity. In one patient, factor XI:C and XII:C activities were reduced to 46% and 53% respectively, and APTT was prolonged to 48.5 seconds. In the other, factor VII:C activity had dropped to 69%, and Normotest results were only 62%. Ten of the 14 patients and 1 woman of the control group were diagnosed as having mild anemia. We conclude that investigations for vWD I should only be done in women with a previous history of abnormal bleeding. Received: 25 April 2001 / Accepted: 20 June 2001     

von Willebrand's disease and pregnancy: management during delivery and outcome of offspring

Six patients having different subtypes of von Willebrand's disease were followed up during eight complete pregnancies. Two additional pregnancies terminated in spontaneous abortions. Five pregnancies ended in cesarean section either because of obstetric problems (three) or electively (two) to avoid infant bleeding. Three deliveries were complicated by vaginal bleeding attributed to von Willebrand's disease, while bleeding during two deliveries had clear obstetric causes. Only two deliveries were associated with no bleeding complications. Five newborn babies were found to have von Willebrand's disease. One of them was born with a head hematoma. Management, which included cryoprecipitate and desmopressin (Stimate), is discussed. It is important to manage each case individually since obstetric parameters and severity of bleeding disorder must be known before treatment is planned.     

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.     

Fetal periventricular hemorrhage in von Willebrand's disease: short review and first case presentation

We present the first case of fetal periventricular hemorrhage due to type IIa Von Willebrand's disease. Discussed are the causal relationships between fetal hemostatic disorders and periventricular hemorrhage, the risks of labor and delivery, and the management with respect to antenatal diagnosis, fetal therapy, and delivery.     

Von Willebrand's disease and pregnancy

Seven new cases of pregnancy combined with von Willebrand's disease have been reported. Altogether the patients had 11 deliveries, and one abortion after 10 weeks' gestation. The diagnosis was based on the determination of the bleeding time, factor VIII coagulant activity (VIII C), factor VIII-related antigen (VIII RAg) and the ristocetin cofactor (VIII RCoF). Two of our 6 parturients showed profuse bleeding at delivery; in addition one patient showed bleeding after abortion at 10 weeks' gestation. Vaginal delivery is generally safe in patients with von Willebrand's disease. There is a great risk of copious bleeding especially in connection with early abortion, when factor VIII levels are low.     

Von Willebrand's disease and pregnancy.

The course of a pregnant patient with von Willebrand's disease, who underwent a successful cesarean section without supportive Factor VIII therapy is described. She had no postoperative bleeding despite a persistently prolonged bleeding time. The significance of the bleeding time, Factor VIII activity, and Factor VIII-related antigen in relation to bleeding tendency and therapeutic management is discussed.     

Severe thrombocytopenia in a pregnant patient with platelet-associated IgM, and known von Willebrand's disease; a case report

In this report, a patient with known von Willebrand's disease is presented with severe thrombocytopenia developing during pregnancy, which at first was believed to be caused by immune thrombocytopenic purpura. For several reasons this first diagnosis had to be rejected, but subtyping of von Willebrand's disease pointed out that the patient suffered from type IIB, which, according to recent literature, can lead to platelet destruction in pregnancy. Early subtyping of von Willebrand's disease can have a major influence on the management of pregnancy.