Table 1.
Patient characteristics
| Characteristics | Type 1 without variant (n = 72) | Type 1 with variant (n = 108) | Type 2 VWD (n = 70) |
|---|---|---|---|
| Age at desmopressin | 37 [26-46] | 35 [18-45] | 38 [27-48] |
| Female, n (%) | 48 (66.7%) | 66 (61.1%) | 39 (55.7%) |
| Blood group O, n (%) | 54 (75.0%)* | 60 (60.6%)* | 38 (55.1%)* |
| Genetic analysis, n (%) | 63 (87.5%) | 86 (79.4%) | 59 (84.3%) |
| Included AFMs, n (%) | 9 (12.5%) | 22 (20.4%) | 11 (15.7%) |
| Bleeding score1 | 9 [5-15]* | 7 [4-12]* | 11 [6-14]* |
| Historically lowest VWF levels2 | |||
| VWF:Ag | 0.39 [0.30-0.46]* | 0.26 [0.15-0.36]* | 0.31 [0.19-0.43]* |
| VWF:Act | 0.24 [0.20-0.27]* | 0.18 [0.10-0.25]* | 0.09 [0.04-0.22]* |
| VWF:CB | 0.25 [0.19-0.33]* | 0.19 [0.10-0.26]* | 0.15 [0.03-0.27]* |
| FVIII:C | 0.50 [0.40-0.61]* | 0.44 [0.27-0.60]* | 0.38 [0.26-0.57]* |
| FVIII:C/VWF:Ag ratio1 | 1.6 [1.4-1.8]* | 2.0 [1.6-2.5]* | 1.5 [1.2-2.0]* |
| VWFpp/VWF:Ag ratio1 | 2.0 [1.7-2.3]* | 2.8 [1.9-5.0]* | 4.1 [3.1-5.6]* |
Data are presented as median [interquartile range] unless otherwise specified. AFMs affected family members. 1FVIII:C/VWF:Ag ratio and VWFpp/VWF:Ag ratio were obtained at the inclusion in the WiN study. 2Historically lowest levels were measured at the local laboratories were also VWF and FVIII levels before and after desmopressin administration were measured.
*
P < .05. between groups.