Table 2.

Differences in Type 1 VWD Patients With and Without a VWF Gene Variant

	With Variant	Without Variant	P
	n = 155	n = 97
Age diagnosis, mean ± SD	26 ± 18	32 ± 14	0.003
Age inclusion, mean ± SD	41 ± 19	48 ± 15	<0.001
Female, n (%)	100 (64.5%)	67 (69.1%)	0.457
Blood group O, n (%)	95 (63.3%)	74 (76.3%)	0.032
Positive family history	114 (91.9%)	37 (56.9%)	<0.001
Reason for referral
Bleeding	71 (49.3%)	69 (75.8%)	<0.001
Family history	73 (50.7%)	22 (24.2%)
VWF:Ag historically lowesta	0.28 (0.20–0.38)	0.40 (0.31–0.46)	0.023
VWF:Ab historically lowesta	0.20 (0.10–0.28)	0.24 (0.20–0.28)	<0.001
VWF:CB historically lowesta	0.21 (0.10–0.30)	0.27 (0.20–0.37)	0.006
FVIII:C historically lowest	0.46 (0.34–0.66)	0.51 (0.40–0.66)	0.288
VWF:Agb	0.27 (0.19–0.39)	0.53 (0.41–0.66)	<0.001
VWF:Abb	0.26 (0.17–0.48)	0.67 (0.53–0.93)	<0.001
VWF:CBb	0.27 (0.17–0.46)	0.62 (0.52–0.86)	<0.001
FVIII:Cb	0.56 (0.39–0.77)	0.86 (0.67–1.05)	<0.001
FVIII:C/VWF:Ag ratioc	1.94 (1.56–2.48)	1.63 (1.43–1.78)	<0.001
VWFpp/VWF:Ag ratiod	2.74 (1.77–4.43)	1.97 (1.60–2.23)	<0.001
Bleeding score	9 (5–14)	10 (6–18)	0.007

Data are presented as median (interquartile range), unless otherwise specified.

^aIn all patients, either historically lowest VWF:Ag, VWF:Ab, or VWF:CB was below 0.30 IU/mL.

^bCentrally measured in the WiN study.

^cNormal ratio <1.9.

^dNormal ratio <2.2. Independent t-test for continuous variables and chi-square for categorical variables.

VWD = von Willebrand disease.