Table 3.
Summary of treatment (including rehabilitation) for 57 major surgical procedures and 67 major bleeding episodes
| Details cohort | N | n | VWF treatment: median (range) | Red Blood cells (n) | ||||
|---|---|---|---|---|---|---|---|---|
| Number of infusions | Number of exposure days | VWF:RCo dose | ||||||
| IU/kg/day | Total IU/kg | |||||||
| Major procedures | 50 | 57 | 13.0 (6‐52) | 10.0 (6‐45) | 44.5 (17‐128) | 476 (134‐1662) | 10 | |
| Category | Oro‐dental | 5 | 5 | 10.0 (6‐17) | 6.0 (6‐12) | 58.1 (17‐128) | 349 (134‐878) | 0 |
| Obs/Gyn | 16 | 19 | 12.0 (8‐23) | 9.0 (6‐16) | 39.6 (18‐83) | 343 (202‐1325) | 1 | |
| Orthopedic | 10 | 12 | 22.5 (9‐39) | 14.0 (6‐35) | 47.4 (23‐93) | 648 (239‐1662) | 2 | |
| Digestive | 7 | 7 | 12.0 (6‐21) | 10.0 (6‐13) | 39.4 (18‐65) | 329 (220‐849) | 4 | |
| General | 8 | 8 | 17.0 (11‐52) | 9.5 (8‐45) | 69.8 (34‐93) | 725 (560‐1513) | 0 | |
| Urological | 3 | 3 | 8.0 (7‐9) | 7.0 (7‐7) | 55.5 (32‐93) | 389 (226‐651) | 0 | |
| Cardiovascular | 2 | 2 | 21.5 (13‐30) | 12.5 (9‐16) | 46.3 (41‐52) | 598 (369‐827) | 2 | |
| Neurological | 1 | 1 | 28.0 | 27.0 | 41.7 | 1127 | 1 | |
| Age at event | <12 years | 7 | 7 | 13.0 (8‐28) | 9.0 (7‐18) | 70.0 (56‐93) | 644 (389‐1396) | 0 |
| 12 to 64 years | 34 | 39 | 13.0 (6‐52) | 10.0 (6‐45) | 41.0 (17‐128) | 430 (134‐1662) | 5 | |
| ≥65 years | 9 | 11 | 12.0 (6‐34) | 9.0 (6‐25) | 42.7 (26‐93) | 349 (226‐1046) | 5 | |
| Major bleeding episodes | 38 | 67 | 8.5 (1‐50)* | 6.0 (1‐47)* | 56.9 (25‐138)* | 390 (44‐2077) | 29 | |
| Location | Gastrointestinal | 18 | 34 | 8.5 (1‐42) | 5.5 (1‐26) | 54.6 (26‐138) | 369 (44‐2077) | 25 |
| Musculoskeletal | 8 | 13 | 10.0 (4‐24) | 8.0 (4‐17) | 55.9 (31‐100) | 495 (215‐1597) | 0 | |
| Genito‐urinary | 7 | 7 | 13.0 (8‐25)* | 9.5 (5‐24)* | 60.3 (49‐72)* | 406 (154‐1176) | 0 | |
| Nasopharynx | 4 | 7 | 5.0 (4‐21) | 4.0 (4‐12) | 76.5 (42‐107) | 308 (168‐1286) | 3 | |
| Intrabuccal | 1 | 1 | 19.0 | 19.0 | 60.9 | 1158 | 1 | |
| Other | 5 | 5 | 17.0 (3‐50) | 11.0 (3‐47) | 58.0 (25‐78) | 603 (233‐1178) | 0 | |
| Age at event | <12 years | 9 | 16 | 6.5 (2‐24) | 5.5 (2‐19) | 64.7 (50‐100) | 388 (106‐1597) | 1 |
| 12 to 64 years † | 26 | 45 | 9.5 (1‐50)* | 6.5 (1‐47)* | 55.2 (25‐138)* | 390 (44‐2077) | 23 | |
| ≥65 years | 4 | 6 | 8.5 (4‐10) | 5.0 (3‐10) | 58.8 (38‐79) | 350 (113‐566) | 5 | |
Other: other bleeds included “peritoneal hemorrhage” (n = 1), “post‐procedural hemorrhage” (n = 1), “tongue biting” (n = 1), “cerebral hematoma” (n = 1), “hemoptysis” revealing tuberculosis in a 16‐year‐old type 3 patient requiring secondary prophylaxis totaling 47 exposure days (n = 1).
Abbreviations: N, number of patients; n, number of clinical events; Obs/Gyn, Obstetrics/Gynecology; VWD, von Willebrand disease; VWF:RCo, von Willebrand factor ristocetin cofactor activity.
Number of infusions and treatment days were missing for one patient experiencing genito‐urinary bleeding episode, aged between 12 to 64 years and with type 2 VWD (basal VWF:RCo: <10 IU/dL and FVIII:C: 31 IU/dL).
One patient changed age group during the study participation and was present in two age groups: 12 to 64 years and ≥ 65 years.