OBSTETRIC MANAGEMENT IN VONWILLEBRANDS-DISEASE - A REPORT OF 24 PREGNANCIES AND A REVIEW OF THE LITERATURE

The case records of 13 patients (24 pregnancies) with von Willebrand's disease (vWD) were studied retrospectively. The overall incidence of primary and secondary post-partum haemorrhage (PPH) was 15.8% and 25% respectively, all primary PPH occurring in type 2 disease (3/14 delive ries, 21.4%). The risk of primary PPH in type 2 patients who did not r eceive prophylactic factor VIII was 37.5% (3/8 deliveries). Factor VII I coagulant activity (VIII:C) and von Willebrand factor antigen (vWF:A g) rose above baseline values by a factor of at least 1.5 during the p regnancy in most cases. More severely affected patients were less like ly to benefit significantly. A baseline VIII:C of <15 iu/dl (4/14 case s) was predictive of a third trimester level of < 15 iu/dl. Improvemen ts in the von Willebrand factor activity were less marked. The baselin e von Willebrand factor activity was <15 iu/dl in all patients with se rial data, none of whom achieved a third-trimester von Willebrand fact or activity of >50 iu/dl. The bleeding times were unaltered significan tly in all but one of the cases, reflecting a general failure of the p rimary haemostatic defect to improve with pregnancy. The findings demo nstrate that coagulation parameters do not universally improve in preg nancy in vWD, especially when preconception levels are low. The risk o f primary PPH is generally higher in type 2 diseases. The level of fac tor VIII:C is not a good predictor of the risk of primary PPH in type 2 patients. Secondary PPH is a significant risk in both type 1 and typ e 2 patients.