Amount of DDAVP and VWF/FVIII concentrates used to treat 232 spontaneous bleeding events in the 75 VWD patients who bled during the 1-y follow-up, according to VWD types and BS
| Bleeders at follow-up . | VWD1, n = 23 . | VWD2A, n = 10 . | VWD2B, n = 5 . | VWD2M, n = 12 . | VWD3, n = 25 . | All VWD, n = 75 . |
|---|---|---|---|---|---|---|
| Total no. of bleeding events (events/pt) | 49 (2.13) | 35 (3.50) | 13 (2.60) | 36 (3.00) | 99 (3.96) | 232 (3.09) |
| No. of bleeds/y in VWD with BS >10 (% by VWD type) | 28 (57) | 19 (54) | 7 (54) | 18 (50) | 84 (85) | 156 (67) |
| Cases treated with DDAVP, no. (%) | 18 (78) | 8 (80) | 0 (0) | 12 (100) | 0 (0) | 38* (51) |
| DDAVP dose,† µg/pt/y, median (range) | 105 (42-252) | 189 (63-305) | 0 (0) | 63 (21-345) | 0 (0) | 105 (21-345) |
| DDAVP dose in VWD with BS >10, median (range) | 210 (189-252) | 235 (189-305) | 0 (0) | 302 (251-345) | 0 (0) | 241 (189-345) |
| Cases treated with VWF/FVIII concentrates, no. (%) | 6 (26) | 10 (100) | 5 (100) | 7 (58) | 25 (100) | 53* (71) |
| Concentrate dose‡ of VWF/RCo 1000 U/pt/y, median (range) | 61 (16-256) | 77 (5-126) | 45 (7-93) | 27 (5-75) | 105 (18-235) | 75 (5-256) |
| Concentrate dose in VWD with BS >10, median (range) | 157 (48-256) | 82 (75-126) | 93 | 44 (27-75) | 156 (63-235) | 138 (27-256) |
| Bleeders at follow-up . | VWD1, n = 23 . | VWD2A, n = 10 . | VWD2B, n = 5 . | VWD2M, n = 12 . | VWD3, n = 25 . | All VWD, n = 75 . |
|---|---|---|---|---|---|---|
| Total no. of bleeding events (events/pt) | 49 (2.13) | 35 (3.50) | 13 (2.60) | 36 (3.00) | 99 (3.96) | 232 (3.09) |
| No. of bleeds/y in VWD with BS >10 (% by VWD type) | 28 (57) | 19 (54) | 7 (54) | 18 (50) | 84 (85) | 156 (67) |
| Cases treated with DDAVP, no. (%) | 18 (78) | 8 (80) | 0 (0) | 12 (100) | 0 (0) | 38* (51) |
| DDAVP dose,† µg/pt/y, median (range) | 105 (42-252) | 189 (63-305) | 0 (0) | 63 (21-345) | 0 (0) | 105 (21-345) |
| DDAVP dose in VWD with BS >10, median (range) | 210 (189-252) | 235 (189-305) | 0 (0) | 302 (251-345) | 0 (0) | 241 (189-345) |
| Cases treated with VWF/FVIII concentrates, no. (%) | 6 (26) | 10 (100) | 5 (100) | 7 (58) | 25 (100) | 53* (71) |
| Concentrate dose‡ of VWF/RCo 1000 U/pt/y, median (range) | 61 (16-256) | 77 (5-126) | 45 (7-93) | 27 (5-75) | 105 (18-235) | 75 (5-256) |
| Concentrate dose in VWD with BS >10, median (range) | 157 (48-256) | 82 (75-126) | 93 | 44 (27-75) | 156 (63-235) | 138 (27-256) |
pt, patient.
Some patients were treated with both DDAVP and VWF/FVIII concentrates.
Each patient was treated with the same dosage of DDAVP equal to 0.3 μg/kg. The median doses of DDAVP calculated in microgram per patient per year are shown also according to BS <5, 5 to 10, >10 by 3 VWD types (see also Figure 4A-C).
Patients have been treated with different dosages (30-80 VWF:RCo U/kg) according to the different sites and severity of bleedings based on the judgment of the investigators. The median doses of VWF/FVIII concentrates expressed in total VWF:RCo IU × 1000/patient per year are shown also according to BS >10 by the 5 VWD types (see also Figure 4A-C).