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Postpartum Hemorrhage in Women with von Willebrand Disease and Carriers of Hemophilia: A Retrospective Analysis

M.C. Punt1, F. van Leusden1, K.W.M. Bloemenkamp2, M. Coppens3, M.H.E. Driessens4, S. Haitjema5, F.C. Heubel-Moenen6,7, A.T. Lely2, A.B. Mäkelburg8, L. Nieuwenhuizen6,9, W.W. van Solinge5, J.L. Saes10,6, S.E.M. Schols10,6, R.E.G. Schutgens1, J. Eikenboom11, M.J. Kruip12, K.P.M. van Galen1

1Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands, 2Department of Obstetrics, Birth Centre Wilhelmina’s Children Hospital, Division Woman and Baby, Utrecht University, Utrecht, Netherlands, 3Department of Vascular Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands, 4Dutch Society of Hemophilia Patients (NVHP), Nijkerk, Netherlands, 5University Medical Center Utrecht, University Utrecht, Laboratory of Clinical Chemistry and Hematology, Utrecht, Netherlands, 6Hemophilia Treatment Center, Nijmegen-Eindhoven-Maastricht, Netherlands, 7Department of Haematology-Internal Medicine, MUMC+, Maastricht, Netherlands, 8Department of Hematology, University Medical Center Groningen, Groningen, Netherlands, 9Department of Thrombosis and Hemostasis, Maxima Medical Center, Veldhoven, Netherlands, 10Radboud University Medical Center, Department of Hematology, Nijmegen, Netherlands, 11Department of Internal Medicine, Division of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, Netherlands, 12Department of Hematology Erasmus MC, Erasmus University Medical Center, Rotterdam, Netherlands

Abstract Number: OC 62.4

Meeting: ISTH 2021 Congress

Theme: Women Health » Pregnancy and Pregnancy Complications

Background: Between 2002-2011, the incidence of severe primary postpartum hemorrhage (PPH) in women with Von Willebrand’s disease (VWD) and hemophilia carriers (HCs) in the Netherlands was 8% (vs. 4.5% in the general population). We hypothesize that PPH has declined over time due to improved management and increased awareness of bleeding disorders.

Aims: To determine the latest incidence of severe primary PPH in women with VWD and HCs in relation to clotting factor activity level and pregnancy management.

Methods: All women with VWD or HCs who delivered between 2012-2017 were selected from all six Dutch Hemophilia Treatment Centers. Patient/disease characteristics, peripartum hematologic/obstetric management and outcomes were retrospectively collected. Severe primary (≥1000 mL blood loss ≤24 hours) and primary (≥500 mL <24hrs) PPH incidence was compared with 1) the previous cohort and 2) the general Dutch population, and between 3) women with third trimester clotting factor activity levels <50 IU/dL vs. ≥50 IU/dL and 4) women treated with vs. without prophylaxis.

Results: 348 deliveries (151 VWD, 167 hemophilia A and 30 hemophilia B carriers) were included. The severe primary PPH incidence was 10% (36/348, Table 1). This risk hasn’t declined over time (prior cohort 8%), but was not higher than seen in the general population (8%, p-value = 0.17). Primary PPH risk was higher in women with coagulation activity levels <50 vs. >50 IU/dL (OR: 2.03, 95% Confidence Interval (CI): 1.16-3.55), despite comparable PPH risk factors. Likewise, women treated with prophylaxis had a higher primary PPH risk than women without (OR: 1.97, 95% CI: 1.18-3.27).

Deliveries
N
Severe primary PPH
N (%)
Primary PPH
N (%)​​​​​
Current cohort Total
   VWD type 1
   VWD type 2
   VWD type 3
   Hemophilia A carrier
   Hemophilia B carrier
348
116
34
1
167
30
36 (10)
14 (12)
6 (18)
0 (0)
13 (8)
3 (10)
99 (28)
36 (31)
17 (50)
1 (100)
34 (20)
11 (37)
Factor level in third trimester
   <50 IU/dL
      Prophylaxis
      No prophylaxis
   ≥50 IU/dL
      Prophylaxis
      No prophylaxis  
66
63
3
279
28
251
7 (11)
7 (11)
0 (0)
29 (10)
4 (14)
25 (10)
27 (41)
27 (43)
0 (0)
71 (25)
9 (32)
62 (25)
Previous Dutch cohort^ 185 14 (8) 62 (34)
General population* 743.591 57.159 (8) NA
Legend: VWD = Von Willebrand’s Disease. PPH = postpartum hemorrhage, severe primary and primary PPH being ≥1000 mL and ≥500 mL blood loss within 24 hours, respectively. ^Data derived from Stoof et al. *Data from Perined. NA= Not available.

Table 1. Postpartum hemorrhage according to bleeding disorder and third trimester factor activity level

Conclusions: Severe primary PPH in women with VWD and HCs did not decline over time and is comparable with the increasing prevalence in the general population. Women with lowest factor levels remain at highest risk for PPH, despite prophylaxis.

To cite this abstract in AMA style:

Punt MC, van Leusden F, Bloemenkamp KWM, Coppens M, Driessens MHE, Haitjema S, Heubel-Moenen FC, Lely AT, Mäkelburg AB, Nieuwenhuizen L, van Solinge WW, Saes JL, Schols SEM, Schutgens REG, Eikenboom J, Kruip MJ, van Galen KPM. Postpartum Hemorrhage in Women with von Willebrand Disease and Carriers of Hemophilia: A Retrospective Analysis [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/postpartum-hemorrhage-in-women-with-von-willebrand-disease-and-carriers-of-hemophilia-a-retrospective-analysis/. Accessed October 1, 2023.

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