Table 2.
Patients’ clinical characteristics by clinical setting (excluding prophylaxis)
| Details cohort | Bleeding episodes | Surgical procedures and childbirths | Invasive procedures | ||
|---|---|---|---|---|---|
| Major | Minor | Major | Minor | ||
| Patients (characteristics at inclusion) | |||||
| Number of patients, N | 83 | 105 | 27 | ||
| 38 | 70 | 50 | 66 | ||
| Male/female | 19/19 | 35/35 | 19/31 | 23/43 | 10/17 |
| Median age (range; years) | 33 (0‐77) | 30 (0‐77) | 32 (2‐83) | 34 (0‐77) | 48 (1‐83) |
| VWF:RCo ≤ 15 IU/dL, N (%) | 35 (92.1) | 66 (94.3) | 36 (72.0) | 45 (68.2) | 23 (85.2) |
| FVIII:C ≤ 40 IU/dL, N (%) | 31 (81.6) | 58 (82.9) | 32 (64.0) | 44 (66.7) | 22 (81.5) |
| FVIII:C < 20 IU/dL, N (%) | 23 (60.5) | 38 (54.3) | 16 (32.0) | 27 (40.9) | 12 (44.4) |
| Events (characteristics at event) | |||||
| Number of clinical events, n | 743 | 140 | 47 | ||
| 67 | 676 | 57 | 83 | ||
| By patient's age | |||||
| <12 years, n (%) | 16 (23.9) | 138 (20.4) | 7 (12.3) | 8 (9.6) | 12 (25.5) |
| 12‐64 years, n (%) | 45 (67.2) | 507 (75.0) | 39 (68.4) | 70 (84.3) | 26 (55.3) |
| ≥65 years, n (%) | 6 (9.0) | 31 (4.6) | 11 (19.3) | 5 (6.0) | 9 (19.1) |
| By VWD type | |||||
| Type 1, n (%) | 5 (7.5) | 49 (7.2) | 12 (21.1) | 21 (25.3) | 8 (17.0) |
| Type 2, n (%) | 36 (53.7) | 298 (44.1) | 34 (59.6) | 47 (56.6) | 21 (44.7) |
| Type 3, n (%) | 25 (37.3) | 328 (48.5) | 10 (17.5) | 12 (14.5) | 18 (38.3) |
| Not classified, n (%) | 1 (1.5) | 1 (0.1) | 1 (1.8) | 3 (3.6) | 0 (0.0) |
| By basal plasma levels | |||||
| VWF:RCo ≤ 15 IU/dL, n (%) | 63 (94.0) | 667 (98.6) | 41 (71.9) | 54 (65.0) | 41 (87.2) |
| FVIII:C ≤ 40 IU/dL, n (%) | 56 (83.6) | 541 (80.0) | 36 (63.1) | 54 (65.0) | 40 (85.1) |
| FVIII:C < 20 IU/dL, n (%) | 40 (59.7) | 429 (63.5) | 17 (29.8) | 31 (37.3) | 22 (46.8) |
| Total number of infusions | 783 | 1462 | 934 | 276 | 103 |
| Total number of exposure days | 608 | 1337 | 676 | 198 | 92 |
Abbreviations: FVIII:C, factor VIII coagulant activity; N, number of patients; n, number of clinical events; VWD, von Willebrand disease; VWF:RCo, von Willebrand factor ristocetin cofactor activity.