Introduction: Women with von Willebrand disease (VWD) are at a higher risk of bleeding, which might affect the health of mother and child during pregnancy and the intra- and postpartum periods. This retrospective cohort study evaluates changes in the coagulation parameters von Willebrand factor antigen (VWF:Ag), von Willebrand ristocetin cofactor (VWF:RCo), and Factor VIII activity (FVIII:C) during pregnancy in patients with VWD. In total, 44 pregnancies of 38 patients were assessed (VWD type 1 n = 32, type 2A n = 3, type 2B n = 1, type 2 subtype unidentified n = 2). The patients’ median age at childbirth was 32 years (range 22–40). Results: A significant increase in coagulation parameters was found in patients with VWD type 1 (VWF:Ag, VWF:RCo, and FVIII:C p = 0.000). In the third trimester, VWF:Ag and FVIII:C normalized in all patients with VWD type 1; in 3 patients VWF:RCo remained below the normal range. Patients with VWD type 2 showed a significant increase of VWF:Ag (p = 0.003) and FVIII:C (p = 0.011), and a non-significant increase of VWF:RCo (p = 0.097). In 4 of 9 pregnancies of patients with VWD type 2, all surveyed coagulation parameters normalized until the third trimester. Conclusion: For the majority of the observed patients, the von Willebrand parameters increased during pregnancy.

1.
De Meyer
SF
,
Deckmyn
H
,
Vanhoorelbeke
K
.
von Willebrand factor to the rescue
.
Blood
.
2009
May
;
113
(
21
):
5049
57
.
[PubMed]
0006-4971
2.
Castaman
G
,
Goodeve
A
,
Eikenboom
J
;
European Group on von Willebrand Disease
.
Principles of care for the diagnosis and treatment of von Willebrand disease
.
Haematologica
.
2013
May
;
98
(
5
):
667
74
.
[PubMed]
0390-6078
3.
James
AH
.
More than menorrhagia: a review of the obstetric and gynaecological manifestations of von Willebrand disease
.
Thromb Res
.
2007
;
120
Suppl 1
:
S17
20
.
[PubMed]
0049-3848
4.
Hellgren
M
.
Hemostasis during normal pregnancy and puerperium
.
Semin Thromb Hemost
.
2003
Apr
;
29
(
2
):
125
30
.
[PubMed]
0094-6176
5.
Rodeghiero
F
,
Tosetto
A
,
Abshire
T
,
Arnold
DM
,
Coller
B
,
James
P
, et al.;
ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group
.
ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders
.
J Thromb Haemost
.
2010
Sep
;
8
(
9
):
2063
5
.
[PubMed]
1538-7933
6.
Huq
FY
,
Kulkarni
A
,
Agbim
EC
,
Riddell
A
,
Tuddenham
E
,
Kadir
RA
.
Changes in the levels of factor VIII and von Willebrand factor in the puerperium.
Haemophilia : the official journal of the World Federation of Hemophilia.
2012
;18(2):241-5.
7.
Nowak-Göttl
U
,
Limperger
V
,
Kenet
G
,
Degenhardt
F
,
Arlt
R
,
Domschikowski
J
, et al.
Developmental hemostasis: A lifespan from neonates and pregnancy to the young and elderly adult in a European white population
.
Blood Cells Mol Dis
.
2017
Sep
;
67
:
2
13
.
[PubMed]
1079-9796
8.
Ewodo
S
,
Nguefack
CT
,
Adiogo
D
,
Etong
EM
,
Beyiha
G
,
Belley
PE
.
[Changes of Von Willebrand factor concentration during pregnancy]
.
Ann Biol Clin (Paris)
.
2014
May-Jun
;
72
(
3
):
292
6
.
[PubMed]
1950-6112
9.
Drury-Stewart
DN
,
Lannert
KW
,
Chung
DW
,
Teramura
GT
,
Zimring
JC
,
Konkle
BA
, et al.
Complex changes in von Willebrand factor-associated parameters are acquired during uncomplicated pregnancy
.
PLoS One
.
2014
Nov
;
9
(
11
):
e112935
.
[PubMed]
1932-6203
10.
Rydz
N
,
Grabell
J
,
Lillicrap
D
,
James
PD
.
Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease.
Haemophilia : the official journal of the World Federation of Hemophilia.
2015
.
11.
Sanders
YV
,
Giezenaar
MA
,
Laros-van Gorkom
BA
,
Meijer
K
,
van der Bom
JG
,
Cnossen
MH
, et al.;
WiN study group
.
von Willebrand disease and aging: an evolving phenotype
.
J Thromb Haemost
.
2014
Jul
;
12
(
7
):
1066
75
.
[PubMed]
1538-7933
12.
Delbrück
C
,
Haferland
I
,
Scholz
K
,
Asmelash
G
,
Makki
V
,
Müller
S
, et al.
[Does FVIII-activity increase with age in patients with haemophilia A and carriers of haemophilia A?]
.
Hamostaseologie
.
2011
Nov
;
31
Suppl 1
:
S24
8
.
[PubMed]
0720-9355
13.
Scholz
U
,
Oppermann
J
,
Siegemund
A
,
Schobess
R
.
[Von Willebrand disease type 1 and pregnancy]
.
Hamostaseologie
.
2011
Nov
;
31
Suppl 1
:
S11
3
.
[PubMed]
0720-9355
14.
Shahbazi
S
,
Moghaddam-Banaem
L
,
Ekhtesari
F
,
Ala
FA
.
Impact of inherited bleeding disorders on pregnancy and postpartum hemorrhage.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis.
2012
;23(7):603-7.
15.
James
AH
,
Jamison
MG
.
Bleeding events and other complications during pregnancy and childbirth in women with von Willebrand disease
.
J Thromb Haemost
.
2007
Jun
;
5
(
6
):
1165
9
.
[PubMed]
1538-7933
16.
James
AH
,
Konkle
BA
,
Kouides
P
,
Ragni
MV
,
Thames
B
,
Gupta
S
, et al.
Postpartum von Willebrand factor levels in women with and without von Willebrand disease and implications for prophylaxis.
Haemophilia : the official journal of the World Federation of Hemophilia.
2015
;21(1):81-7.
17.
Ramsahoye
BH
,
Davies
SV
,
Dasani
H
,
Pearson
JF
.
Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a review of the literature.
Haemophilia : the official journal of the World Federation of Hemophilia.
1995
;1:140-4.
18.
Gynecologists ACoOa
.
Von Willebrand’s disease in women. Committee opinion No.580
.
Obstet Gynecol
.
2013
;
122
:
1368
73
. 0029-7844
19.
Castaman
G
,
Tosetto
A
,
Rodeghiero
F
.
Pregnancy and delivery in women with von Willebrand’s disease and different von Willebrand factor mutations
.
Haematologica
.
2010
Jun
;
95
(
6
):
963
9
.
[PubMed]
0390-6078
20.
Mannucci
PM
.
Treatment of von Willebrand’s Disease
.
N Engl J Med
.
2004
Aug
;
351
(
7
):
683
94
.
[PubMed]
0028-4793
21.
Castaman
G
.
Changes of von Willebrand Factor during Pregnancy in Women with and without von Willebrand Disease
.
Mediterr J Hematol Infect Dis
.
2013
Jul
;
5
(
1
):
e2013052
.
[PubMed]
2035-3006
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