Table 2.
Surgical procedures and perioperative factor replacement therapy in patients with severe and mild rare bleeding disorders. Only cases in which postoperative bleeding was excessive are listed.
| Surgical interventions in patients with severe coagulation factor deficiency (factor level ≤10%). | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Factor deficiency | Total | Perioperative factor replacement | N (%) | Factor level median (min-max) |
Age (y), median (min-max) | Tosetto’s score, median (min;max) | High risk | Low risk | Surgeries with bleeding (%) |
| FVII | 51 | Yes | 39 (76.5) | 4 (0-10) | 48 (0-84) | 4 (-1;34) | 24 | 15 | 1 (2.5) |
| No | 12 (23.5) | 6.5 (2-10) | 40 (0-84) | 4(-1;7) | 3 | 9 | 0 | ||
| FXI | 24 | Yes | 11 (45.8) | 4.5 (0-10) | 55 (17-86) | 1(-2;15) | 6 | 5 | 1 (9.9) |
| No | 13 (54.2) | 1 (0-10) | 31 (22-70) | 1(-2;8) | 3 | 10 | 0 | ||
| FV | 1 | Yes | 0 | NA | NA | NA | NA | NA | NA |
| No | 1 (100) | NA | NA | NA | 0 | 1 | 0 | ||
| FV+FVIII | 2 | Yes | 2 (100) | 9 (8-10) | NA | NA | 2 | 0 | 0 |
| No | 0 | NA | NA | NA | NA | NA | NA | ||
| FX | 3 | Yes | 2 (66.7) | 2 (2-2) | NA | NA | 1 | 1 | 0 |
| No | 1 (33.3) | NA | NA | NA | 0 | 1 | 0 | ||
| Total | 81 | Yes | 54 (66.7) | 4 (0-10) | 55 (0-86) | 4(-2;34) | 33 | 21 | 2 (3.7) |
| No | 27 (33.3) | 2 (0-10) | 35 (0-84) | 4(-2;16) | 6 | 21 | 0 | ||
| Surgical interventions in patients with mild coagulation factor deficiency (factor level >10%) | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Factor deficiency | Total | Perioperative factor replacement | N (%) | Factor level median (min-max) |
Age (y), median (min-max) | Tosetto’s score, median (min;max) | High risk | Low risk | Surgeries with bleeding (%) |
| FVII | 67 | Yes | 15 (22.4) | 18 (12-39) | 32 (6-78) | 2(-3;12) | 10 | 5 | 0 |
| No | 52 (77.6) | 29 (11-46) | 26 (0-78) | 3(-3;10) | 26 | 26 | 1 (1.9) | ||
| FXI | 40 | Yes | 8 (20) | 29.5 (14-41) | 35 (14-74) | 2(0;10) | 5 | 3 | 0 |
| No | 32 (80) | 33.5 (14-42) | 31 (4-82) | 0(-3;6) | 17 | 15 | 0 | ||
| FII | 1 | Yes | 0 | NA | NA | NA | NA | NA | NA |
| No | 1 (100) | NA | NA | NA | 1 | 0 | 0 | ||
| FV | 13 | Yes | 0 | NA | NA | NA | NA | NA | NA |
| No | 13 (100) | 35 (24-47) | 21 (1-37) | 0(-1;5) | 6 | 7 | 0 | ||
| FV+FVIII | 1 | Yes | 0 | NA | NA | NA | NA | NA | NA |
| No | 1 (100) | NA | NA | NA | 0 | 1 | 0 | ||
| FX | 4 | Yes | 3 (75) | 11 (11-33) | NA | NA | 2 | 1 | 0 |
| No | 1 (25) | NA | NA | NA | 0 | 1 | 0 | ||
| Total | 126 | Yes | 26 (20.6) | 23 (11-41) | 35 (6-78) | -1(-3;12) | 17 | 9 | 0 |
| No | 100 (79.4) | 31 (11-49) | 27 (0-82) | 0(-3;10) | 50 | 50 | 1 (1.0) | ||