Demographic and clinical-laboratory features at baseline of the entire cohort (n = 796) vs bleeders (n = 75) of the VWD patients according to types
| . | VWD1 . | VWD2A . | VWD2B . | VWD2M . | VWD3 . | All VWD types . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| All cases, n = 457 . | Bleeders, n = 23 . | All cases, n = 65 . | Bleeders, n = 10 . | All cases, n = 56 . | Bleeders, n = 5 . | All cases, n = 169 . | Bleeders, n = 12 . | All cases, n = 49 . | Bleeders, n = 25 . | All cases, n = 796 . | Bleeders, n = 75 . | |
| Gender, M/F | 183/274 | 10/13 | 31/34 | 7/3 | 23/33 | 3/2 | 75/94 | 6/6 | 21/28 | 11/14 | 333/463 | 37/38 |
| Age, median (range), y | 39 (6-99) | 39 (13-67) | 39 (7-81) | 59 (11-85) | 37 (8-84) | 45 (16-61) | 39 (10-89) | 37 (18-73) | 29 (5-60) | 33 (10-61) | 38 (5-99) | 37 (10-85) |
| BS, median (range) | 4 (−3-28) | 6 (2-28) | 8 (1-19) | 9 (3-19) | 6 (0-24) | 7 (1-24) | 5 (0-28) | 4 (0-28) | 14 (3-35) | 16 (5-35) | 5 (−3-35) | 9 (0-35) |
| VWF:RCo, median (range), IU/dL | 31 (3-56) | 25 (6-56) | 6 (2-53) | 6 (2-22) | 20 (6-79) | 12 (6-23) | 10 (4-50) | 10 (6-37) | <2 (—) | <2 (—) | 17 (<2-79) | 6 (<2-56) |
| <10 IU/dL, case no. (%) | 128 (28) | 8 (35) | 45 (69) | 6 (60) | 8 (14) | 2 (40) | 77 (46) | 5 (42) | 49 (100) | 25 (100) | 307 (38) | 46 (61) |
| 10-30 IU/dL, case no. (%) | 94 (21) | 12 (52) | 18 (28) | 4 (40) | 38 (68) | 3 (60) | 71 (42) | 4 (33) | 0 | 0 | 221 (28) | 16 (21) |
| >30 IU/dL, case no. (%) | 235 (51) | 3 (13) | 2 (3) | 0 | 10 (18) | 0 | 21 (12) | 3 (25) | 0 | 0 | 268 (34) | 13 (17) |
| FVIII:C, median (range), IU/dL | 47 (2-187) | 36 (13-129) | 48 (17-140) | 43 (21-110) | 46 (16-140) | 47 (35-57) | 43 (7-144) | 33 (22-48) | 3 (1-26) | 3 (1-19) | 44 (1-187) | 29 (1-129) |
| Previous DDAVP, case no. (%) | 204 (45) | 23 (100) | 11 (17) | 9 (90) | 5 (9) | 1 (20) | 54 (44) | 9 (75) | 0 | 0 | 274 (34) | 42 (56) |
| Previous conc., case no. (%) | 146 (32) | 11 (48) | 55 (85) | 10 (100) | 35 (62) | 5 (100) | 106 (63) | 10 (83) | 46 (94) | 25 (100) | 388 (49) | 61 (81) |
| . | VWD1 . | VWD2A . | VWD2B . | VWD2M . | VWD3 . | All VWD types . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| All cases, n = 457 . | Bleeders, n = 23 . | All cases, n = 65 . | Bleeders, n = 10 . | All cases, n = 56 . | Bleeders, n = 5 . | All cases, n = 169 . | Bleeders, n = 12 . | All cases, n = 49 . | Bleeders, n = 25 . | All cases, n = 796 . | Bleeders, n = 75 . | |
| Gender, M/F | 183/274 | 10/13 | 31/34 | 7/3 | 23/33 | 3/2 | 75/94 | 6/6 | 21/28 | 11/14 | 333/463 | 37/38 |
| Age, median (range), y | 39 (6-99) | 39 (13-67) | 39 (7-81) | 59 (11-85) | 37 (8-84) | 45 (16-61) | 39 (10-89) | 37 (18-73) | 29 (5-60) | 33 (10-61) | 38 (5-99) | 37 (10-85) |
| BS, median (range) | 4 (−3-28) | 6 (2-28) | 8 (1-19) | 9 (3-19) | 6 (0-24) | 7 (1-24) | 5 (0-28) | 4 (0-28) | 14 (3-35) | 16 (5-35) | 5 (−3-35) | 9 (0-35) |
| VWF:RCo, median (range), IU/dL | 31 (3-56) | 25 (6-56) | 6 (2-53) | 6 (2-22) | 20 (6-79) | 12 (6-23) | 10 (4-50) | 10 (6-37) | <2 (—) | <2 (—) | 17 (<2-79) | 6 (<2-56) |
| <10 IU/dL, case no. (%) | 128 (28) | 8 (35) | 45 (69) | 6 (60) | 8 (14) | 2 (40) | 77 (46) | 5 (42) | 49 (100) | 25 (100) | 307 (38) | 46 (61) |
| 10-30 IU/dL, case no. (%) | 94 (21) | 12 (52) | 18 (28) | 4 (40) | 38 (68) | 3 (60) | 71 (42) | 4 (33) | 0 | 0 | 221 (28) | 16 (21) |
| >30 IU/dL, case no. (%) | 235 (51) | 3 (13) | 2 (3) | 0 | 10 (18) | 0 | 21 (12) | 3 (25) | 0 | 0 | 268 (34) | 13 (17) |
| FVIII:C, median (range), IU/dL | 47 (2-187) | 36 (13-129) | 48 (17-140) | 43 (21-110) | 46 (16-140) | 47 (35-57) | 43 (7-144) | 33 (22-48) | 3 (1-26) | 3 (1-19) | 44 (1-187) | 29 (1-129) |
| Previous DDAVP, case no. (%) | 204 (45) | 23 (100) | 11 (17) | 9 (90) | 5 (9) | 1 (20) | 54 (44) | 9 (75) | 0 | 0 | 274 (34) | 42 (56) |
| Previous conc., case no. (%) | 146 (32) | 11 (48) | 55 (85) | 10 (100) | 35 (62) | 5 (100) | 106 (63) | 10 (83) | 46 (94) | 25 (100) | 388 (49) | 61 (81) |
Bleeders were defined as those VWD patients who had at least 1 spontaneous bleeding event requiring treatment during the 1-y follow-up.
conc., concentrates; F, female; M, male.