Baseline characteristics of the RBiN study population
| RBD deficiency . | All patients . | Women . | Men . | Children . | Median age (range), y . | Median coagulation factor level (range) . | Patients receiving prophylaxis . | Patients with known genotype . |
|---|---|---|---|---|---|---|---|---|
| Fibrinogen | 41 (16) | 22 (54) | 11 (27) | 8 (20) | 38 (4-87) | 700 mg/L (0-3180 mg/L) | 5 (13) | 21 (51) |
| FII | 16 (6) | 7 (44) | 6 (38) | 3 (19) | 37 (10-77) | 53% (0%-66%) | 0 (0) | 5 (31) |
| FV | 25 (10) | 11 (44) | 8 (32) | 6 (24) | 36 (2-73) | 35% (0%-60%) | 1 (4) | 4 (16) |
| FV Amsterdam | 2 (1) | 1 (50) | 1 (50) | 0 | 47.5 (31-64) | 283% (271%-295%) | 0 (0) | 2 (100) |
| FV + FVIII | 6 (2) | 1 (17) | 1 (17) | 4 (67) | 11 (1-35) | 10% (2%-24%) | 0 (0) | 5 (83) |
| FVII | 57 (22) | 21 (37) | 19 (33) | 17 (30) | 32 (2-82) | 30% (0%-61%) | 2 (4) | 6 (11) |
| FX | 8 (3) | 5 (63) | 1 (13) | 2 (25) | 29 (11-58) | 33% (0%-55%) | 1 (14) | 2 (25) |
| FXI | 43 (16) | 22 (51) | 11 (26) | 10 (23) | 41 (2-84) | 35% (0%-58%) | 0 (0) | 4 (9) |
| FXIII | 14 (5) | 2 (14) | 8 (57) | 4 (29) | 31.5 (5-60) | 0% (0%-82%) | 12 (86) | 10 (71) |
| α2-AP | 23 (9) | 13 (57) | 6 (26) | 4 (17) | 50 (6-76) | 61% (0%-82%) | 0 (0) | 5 (21) |
| PAI-1 | 14 (5) | 14 (100) | 0 (0) | 0 (0) | 45 (20-60) | 2.5 ng/mL (1.5-3.2 ng/mL) | 0 (0) | 0 (0) |
| Hyperfibrinolysis | 14 (5) | 12 (86) | 2 (14) | 0 (0) | 49.5 (22-75) | 8.6% (5.8%-12.7%) | 0 (0) | 0 (0) |
| Total | 263 | 131 (50) | 74 (28) | 58 (22) | 38.5 (1-87) | NA | 21 (8) | 69 (26) |
| RBD deficiency . | All patients . | Women . | Men . | Children . | Median age (range), y . | Median coagulation factor level (range) . | Patients receiving prophylaxis . | Patients with known genotype . |
|---|---|---|---|---|---|---|---|---|
| Fibrinogen | 41 (16) | 22 (54) | 11 (27) | 8 (20) | 38 (4-87) | 700 mg/L (0-3180 mg/L) | 5 (13) | 21 (51) |
| FII | 16 (6) | 7 (44) | 6 (38) | 3 (19) | 37 (10-77) | 53% (0%-66%) | 0 (0) | 5 (31) |
| FV | 25 (10) | 11 (44) | 8 (32) | 6 (24) | 36 (2-73) | 35% (0%-60%) | 1 (4) | 4 (16) |
| FV Amsterdam | 2 (1) | 1 (50) | 1 (50) | 0 | 47.5 (31-64) | 283% (271%-295%) | 0 (0) | 2 (100) |
| FV + FVIII | 6 (2) | 1 (17) | 1 (17) | 4 (67) | 11 (1-35) | 10% (2%-24%) | 0 (0) | 5 (83) |
| FVII | 57 (22) | 21 (37) | 19 (33) | 17 (30) | 32 (2-82) | 30% (0%-61%) | 2 (4) | 6 (11) |
| FX | 8 (3) | 5 (63) | 1 (13) | 2 (25) | 29 (11-58) | 33% (0%-55%) | 1 (14) | 2 (25) |
| FXI | 43 (16) | 22 (51) | 11 (26) | 10 (23) | 41 (2-84) | 35% (0%-58%) | 0 (0) | 4 (9) |
| FXIII | 14 (5) | 2 (14) | 8 (57) | 4 (29) | 31.5 (5-60) | 0% (0%-82%) | 12 (86) | 10 (71) |
| α2-AP | 23 (9) | 13 (57) | 6 (26) | 4 (17) | 50 (6-76) | 61% (0%-82%) | 0 (0) | 5 (21) |
| PAI-1 | 14 (5) | 14 (100) | 0 (0) | 0 (0) | 45 (20-60) | 2.5 ng/mL (1.5-3.2 ng/mL) | 0 (0) | 0 (0) |
| Hyperfibrinolysis | 14 (5) | 12 (86) | 2 (14) | 0 (0) | 49.5 (22-75) | 8.6% (5.8%-12.7%) | 0 (0) | 0 (0) |
| Total | 263 | 131 (50) | 74 (28) | 58 (22) | 38.5 (1-87) | NA | 21 (8) | 69 (26) |
All data are n (%), unless otherwise stated. The columns for women and men show the number of patients aged 18 years or older, and the column for children shows patients younger than 18 years old. For patients with FV Amsterdam, the reported value in the column for coagulation factor activity level is the TFPI level. For patients with hyperfibrinolysis, the reported value in the column for coagulation factor level is the euglobulin clot lysis time ratio.
NA, not applicable.