Abstract
Introductionvon Willebrand disease (VWD) is a hereditary bleeding disorder, caused by a deficiency in the levels and/or function of von Willebrand factor (VWF). Women with VWD appear to be at increased risk of experiencing postpartum hemorrhage (PPH), though the levels of VWF increase during pregnancy. There is limited knowledge of how PPH is associated with the subtype of VWD, plasma levels of other coagulations factors than VWF and given hemostatic treatment.AimsThe aims were to investigate the incidence of PPH in women with VWD and to analyse the correlation between PPH and: (1) type of VWD, (2) laboratory monitoring of VWF and FVIII and (3) hemostatic drug treatment.MethodsThis was a retrospective observational study. The study participants (n = 34) were recruited from the Coagulation Unit, Karolinska University hospital. Fifty-nine deliveries, which occurred in 14 different obstetrics units (years 1995–2012) were included in the study.ResultsThe incidence of primary PPH was 44%, severe primary PPH 20% and secondary PPH 12%. VWD type 3 was associated with a higher risk of experiencing severe primary PPH compared to other subtypes. FVIII:C in pregnancy was inversely correlated to blood loss during delivery. There was a significantly higher incidence of secondary PPH when the VWD diagnosis was unknown at time of delivery.ConclusionsThe women with VWD are at higher risk of PPH, especially those with type 3 VWD or when diagnosis is unknown prior to delivery. Identification of pregnant women with undiagnosed VWD may be of importance in order to prevent PPH.
Highlights
Introduction vonWillebrand disease (VWD) is a hereditary bleeding disorder, caused by a deficiency in the levels and/or function of von Willebrand factor (VWF)
Women with Von Willebrand disease (VWD) appear to be at increased risk of experiencing postpartum hemorrhage (PPH), though the levels of VWF increase during pregnancy
VWD type 3 was associated with a higher risk of experiencing severe primary PPH compared to other subtypes
Summary
Willebrand disease (VWD) is a hereditary bleeding disorder, caused by a deficiency in the levels and/or function of von Willebrand factor (VWF). There is limited knowledge of how PPH is associated with the subtype of VWD, plasma levels of other coagulations factors than VWF and given hemostatic treatment. Von Willebrand disease (VWD) is a hereditary bleeding disorder first described in 1926 by Erik von Willebrand [1]. VWD is caused by deficiency of von Willebrand factor (VWF). The prevalence of VWD varies depending on calculation: epidemiological studies report it to be approximately 0.5–1% [5, 6], making VWD the most common inherited bleeding disorder, whereas the prevalence based on the referral rate of VWD patients to specialized coagulation centres is reported to be 0.002–0.01% [7]. In the Nordic region, the prevalence of VWD is approximately 0.008% based on referral rate [8]
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