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) |
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.