VWF parameters and ratios per type of VWD
| VWD type . | No. of Patients . | VWF:Ag (IU/dL) . | VWF:Act (U/dL) . | FVIII:C (IU/dL) . | VWFpp (U/dL) . | VWF:Act/VWF:Ag ratio . | VWFpp/VWF:Ag ratio . | FVIII:C/VWF:Ag ratio . | |||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | ||
| 1* | 381 | 38 | 23-53 | 46 | 24-72 | 67 | 49-88 | 91 | 68-116 | 1.2 | 1.0-1.4 | 2.2 | 1.7-3.1 | 1.8 | 1.4-2.3 |
| 2 | 240 | 24 | 16-34 | 8 | 4-16 | 37 | 27-48 | 104 | 81-136 | 0.4 | 0.2-0.5 | 4.5 | 3.2-6.0 | 1.6 | 1.2-2.0 |
| 2A | 157 | 22 | 14-32 | 8 | 3-15 | 37 | 27-47 | 99 | 79-123 | 0.4 | 0.2-0.5 | 4.5 | 3.3-6.2 | 1.7 | 1.3-2.2 |
| 2B | 42 | 28 | 23-40 | 8 | 6-14 | 39 | 28-48 | 137 | 106-157 | 0.3 | 0.2-0.4 | 4.6 | 3.7-5.8 | 1.3 | 1.1-1.5 |
| 2M | 27 | 24 | 16-30 | 5 | 3-13 | 42 | 31-52 | 93 | 75-119 | 0.3 | 0.1-0.4 | 4.8 | 3.1-6.2 | 1.9 | 1.6-2.1 |
| 2N | 14 | 34 | 28-44 | 45 | 29-54 | 19 | 13-28 | 111 | 71-157 | 1.2 | 1.0-1.5 | 2.7 | 2.0-4.4 | 0.6 | 0.4-0.7 |
| 3 | 37 | 1 | 0-4 | 0 | 0-1 | 3 | 1-9 | 0 | 0-60† | NA | NA | NA | |||
| Normal range‡ | 47-169 | 45-213 | 57-185 | 82-173 | 0.6-1.8 | 0.8-2.2 | 0.6-1.9 | ||||||||
| VWD type . | No. of Patients . | VWF:Ag (IU/dL) . | VWF:Act (U/dL) . | FVIII:C (IU/dL) . | VWFpp (U/dL) . | VWF:Act/VWF:Ag ratio . | VWFpp/VWF:Ag ratio . | FVIII:C/VWF:Ag ratio . | |||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | Median . | IQR . | ||
| 1* | 381 | 38 | 23-53 | 46 | 24-72 | 67 | 49-88 | 91 | 68-116 | 1.2 | 1.0-1.4 | 2.2 | 1.7-3.1 | 1.8 | 1.4-2.3 |
| 2 | 240 | 24 | 16-34 | 8 | 4-16 | 37 | 27-48 | 104 | 81-136 | 0.4 | 0.2-0.5 | 4.5 | 3.2-6.0 | 1.6 | 1.2-2.0 |
| 2A | 157 | 22 | 14-32 | 8 | 3-15 | 37 | 27-47 | 99 | 79-123 | 0.4 | 0.2-0.5 | 4.5 | 3.3-6.2 | 1.7 | 1.3-2.2 |
| 2B | 42 | 28 | 23-40 | 8 | 6-14 | 39 | 28-48 | 137 | 106-157 | 0.3 | 0.2-0.4 | 4.6 | 3.7-5.8 | 1.3 | 1.1-1.5 |
| 2M | 27 | 24 | 16-30 | 5 | 3-13 | 42 | 31-52 | 93 | 75-119 | 0.3 | 0.1-0.4 | 4.8 | 3.1-6.2 | 1.9 | 1.6-2.1 |
| 2N | 14 | 34 | 28-44 | 45 | 29-54 | 19 | 13-28 | 111 | 71-157 | 1.2 | 1.0-1.5 | 2.7 | 2.0-4.4 | 0.6 | 0.4-0.7 |
| 3 | 37 | 1 | 0-4 | 0 | 0-1 | 3 | 1-9 | 0 | 0-60† | NA | NA | NA | |||
| Normal range‡ | 47-169 | 45-213 | 57-185 | 82-173 | 0.6-1.8 | 0.8-2.2 | 0.6-1.9 | ||||||||
IQR, 25% to 75% interquartile range; NA, not applicable.
Median and 25% to 75% IQRs of VWFpp level and ratios in type 1 VWD patients are in accordance with the results from Eikenboom et al.12
VWFpp levels below the assay detection limit (<4 U/dL) were considered 0 (n = 25).
The normal reference ranges (2.5th to 97.5th percentile) are based on 387 healthy controls and were taken from our previous publication Eikenboom et al12 ; however, VWF activity in that study was based on VWF:RCo. VWFpp in the WiN study was measured in this same laboratory.