Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a reivesw of the literature

Abstract

The case records of 13 patients (24 pregnancies) with von Willebrand's disease (vWD) were studies rettospectively. The overall incidence of primary and secondary post-partum haemorrhage (PPH) was 15.8% and 25% respectively, all primary PPH occurring in tyre 2 discase (3/14 deliveries, 21.4%). The risk of primary PPH in type 2 patients who did not receive prophylactic factor VIII was 37.5% (3/8 deliveries). Factor VIII coagulant activity (VIII:C) and von Willebrand factor antigen (vWF:Ag) rose above bascline values by a factor of at least 1.5 during the pregnancy in most case. More severely affected patients were less likely to benefit significatntly. A baseline VIII:C of <15 iu/dl (4/14 cases) was predictive of a third trimester level of <15 iu/dl. Improvements in the von Willebrand factor activity were less marked. The baseline von Willebrand factor activity was <15 iu/dl in all patients with serial data, none of whom achieved a third-trimester von Willebrand factor activity of >50 iu/dl. The bleeding times were unaltered significantly in all but one of the cases, reflecting a general failure of the primary haemostatic defect to improve with pregnancy. The findings demonstrate that coagulation parameters do not universally improve in pregnancy in vWD, especially when preconception levels are low. The risk of primary PPH is generally higher in type 2 diseases. The level of factor VIII:C is not a good predictor of the risk of primary PPH in type 2 patients. Secondary PPH is a significatnt risk in both type 1 and type 2 patients.

Pregnancy and delivery in women with von Willebrand's disease and different von Willebrand factor mutations

“…21 In our study, two women with compound heterozygosity and a severe phenotype had a peculiar pattern. These women had an isolated, significant increase of FVIII:C alone during pregnancy, which was almost similar to that observed after the test infusion of desmopressin (Figure 2) in the woman with Q77/IVS13 mutations.…”

How I treat type 2 variant forms of von Willebrand disease

“…Primary postpartum bleeding is observed in up to 37.5% of type 2 women not receiving FVIII prophylaxis, 68 and special care should be taken to avoid bleeding. This is particularly relevant in the 2 presented patients because of their lifelong history of mucous bleeding, suggesting greater bleeding risk (see "The relevance of bleeding history in guiding the optimal therapy" in this study).…”

Management of Inherited Bleeding Disorders in Pregnancy

“…105 There is usually poor increment in women with a basal level less than 0.15-0.2 iu/ml. 89,106 In type 2A VWD and type 2M VWD during pregnancy, the defect does not correct. A significant increase of factor VIII and VWF antigen occurs, but VWF activity remains markedly reduced.…”

“…However, in patients with severe VWF deficiency, for example, a baseline VWF level of less than 0.15 iu/ml, VWF and factor VIII levels may fail to normalise. 89 In patients with type 2 VWD who exhibit qualitative abnormalities of VWF, the abnormalities may not correct and may even worsen. 90 This is especially the case in type 2B VWD where thrombocytopenia, with its attendant increase in bleeding risk, may be aggravated by the rise in dysfunctional VWF.…”