Table 2

VWF laboratory testing in VWD cohorts

Type 1 VWD cohort (n = 140)Historical type 1 VWD cohort (n = 172)Healthy control cohort (n = 257)
VWF:Ag (IU/dL) 36 (17-46) 76 (64-96) 104 (85-143) 
VWF:RCo (IU/dL) 33 (19-44) 72 (61-97) 100 (76-141) 
VWF:RCo/VWF:Ag ratio 0.98 (0.84-1.13) 1.00 (0.87-1.11) 0.96 (0.84-1.08) 
No. with normal multimer distribution (%) 285 (92) 163 (95) 255 (99) 
VWF:GPIbM 40 (19-54) 94 (75-121) 108 (80-142) 
VWFpp 53 (39-66) 74 (66-88) 88 (75-102) 
VWFpp/VWF:Ag ratio 1.50 (1.23-2.28) 0.98 (0.79-1.15) 0.80 (0.63-0.99) 
FVIII:C 53 (38-71) 85 (73-101) 102 (84-125) 
VWF:CB3 39 (20-52) 83 (72-104) 121 (3-167) 
VWF:CB4 31 (19-42) 71 (56-99) 108 (74-163) 
No. with sequence variants in VWF (%) 193 (62) 24 (14) 36 (14) 
BS 5 (3-8) 5 (3-9) 1 (0-2) 
Type 1 VWD cohort (n = 140)Historical type 1 VWD cohort (n = 172)Healthy control cohort (n = 257)
VWF:Ag (IU/dL) 36 (17-46) 76 (64-96) 104 (85-143) 
VWF:RCo (IU/dL) 33 (19-44) 72 (61-97) 100 (76-141) 
VWF:RCo/VWF:Ag ratio 0.98 (0.84-1.13) 1.00 (0.87-1.11) 0.96 (0.84-1.08) 
No. with normal multimer distribution (%) 285 (92) 163 (95) 255 (99) 
VWF:GPIbM 40 (19-54) 94 (75-121) 108 (80-142) 
VWFpp 53 (39-66) 74 (66-88) 88 (75-102) 
VWFpp/VWF:Ag ratio 1.50 (1.23-2.28) 0.98 (0.79-1.15) 0.80 (0.63-0.99) 
FVIII:C 53 (38-71) 85 (73-101) 102 (84-125) 
VWF:CB3 39 (20-52) 83 (72-104) 121 (3-167) 
VWF:CB4 31 (19-42) 71 (56-99) 108 (74-163) 
No. with sequence variants in VWF (%) 193 (62) 24 (14) 36 (14) 
BS 5 (3-8) 5 (3-9) 1 (0-2) 

Results are given as median (interquartile range). For VWF:RCo, the lower limit of detection is 10 IU/dL. Therefore levels <10 IU/dL were given an average value of 5 IU/dL for calculation of means, with the underlying assumption that levels below 10 would be normally distributed.

FVIII:C, factor VIII activity.

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