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. 2024 Jul 14;8(5):102508.
doi: 10.1016/j.rpth.2024.102508. eCollection 2024 Jul.

Primary postpartum hemorrhage in women with von Willebrand disease and carriers of hemophilia: a retrospective analysis

Marieke Punt  1 , Fe van Leusden  1 , Kitty Bloemenkamp  2 , Michiel Coppens  3   4 , Mariette Driessens  5 , Floor Heubel-Moenen  6   7 , Titia Lely  2 , Anja Mäkelburg  8 , Laurens Nieuwenhuizen  7   9 , Saskia Haitjema  10 , Wouter van Solinge  10 , Joline Saes  7   11 , Saskia Schols  7   11 , Roger Schutgens  1 , Jeroen Eikenboom  12 , Marieke Kruip  13 , Karin van Galen  1
Affiliations

Primary postpartum hemorrhage in women with von Willebrand disease and carriers of hemophilia: a retrospective analysis

Marieke Punt et al. Res Pract Thromb Haemost. .

Abstract

Background: Between 2002 and 2011, the incidence of severe primary postpartum hemorrhage (PPH) in Dutch women with von Willebrand disease (VWD) and hemophilia carriers (HCs) was 8% vs 4.5% in the general population.

Objectives: To determine the contemporary incidence of severe primary PPH in women with VWD and HCs.

Methods: All women with VWD or HCs who delivered between 2012 and 2017 were selected from all 6 Dutch hemophilia treatment centers. Data on patient and disease characteristics, peripartum hematologic and obstetric management, and outcomes were retrospectively collected. Incidence of severe primary (≥1000 mL of blood loss ≤24 hours after childbirth) and primary (≥500 mL within ≤24 hours after childbirth) PPH was compared with the (1) previous cohort and (2) general Dutch population and between (3) women with VWD and HCs with third-trimester coagulation activity levels <50 international units (IU)/dL vs ≥50 IU/dL and (4) women treated with vs without peripartum hemostatic prophylaxis.

Results: Three-hundred forty-eight deliveries (151 VWD, 167 hemophilia A, and 30 hemophilia B carriers) were included. The severe primary PPH incidence was 10% (36/348) and remained stable over time, whereas this incidence has increased in the general population (to 8%), leading to a similar risk (P = .17). Severe primary PPH risk was comparable between women with coagulation activity levels <50 and ≥50 IU/dL (11% [7/66] vs 10% [29/279]; odds ratio, 1.02; 95% CI, 0.43-2.44) and comparable between those with and those without prophylaxis (12% [11/91] vs 10% [25/254]; odds ratio, 1.26; 95% CI, 0.59-2.68).

Conclusion: Severe primary PPH in women with VWD and HCs remained stable and is comparable with the increasing prevalence in the general population. More research is needed to find the optimal pregnancy management strategy for safe delivery in VWD and HC.

Keywords: hemophilia A; hemophilia B; postpartum hemorrhage; pregnancy; von Willebrand diseases.

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