Bleeding symptoms in HA and HB carriers according to minimum coagulation factor levels
| . | FVIII level . | FIX level . | ||||
|---|---|---|---|---|---|---|
| <40 IU/dL . | 40-60 IU/dL . | >60 IU/dL . | <40 IU/dL . | 40-60 IU/dL . | >60 IU/dL . | |
| HMB∗ | ||||||
| Frequency, n (%) | 22/35 (62.9) | 20/48 (41.7) | 23/62 (37.1) | 8/18 (44.4) | 5/16 (31.3) | 10/29 (34.5) |
| RR (95% CI) | 1.96 (1.12-3.42) | 1.11 (0.73-1.71) | 1 | 1.29 (0.63-2.65) | 0.91 (0.39-2.14) | 1 |
| RTB∗,† | ||||||
| Frequency, n (%) | 2/35 (5.7) | 5/48 (10.4) | 7/62 (11.3) | 7/18 (38.9) | 2/16 (12.5) | 3/29 (10.3) |
| RR (95% CI) | 0.59 (0.17-2.07) | 0.95 (0.47-1.92) | 1 | 2.36 (1.24-4.47) | 1.14 (0.36-3.62) | 1 |
| Easy bruising | ||||||
| Frequency, n (%) | 16/38 (42.1) | 20/50 (40.0) | 17/66 (25.8) | 5/22 (22.7) | 3/18 (16.7) | 6/32 (18.8) |
| RR (95% CI) | 1.57 (0.96-2.57) | 1.42 (0.95-2.14) | 1 | 1.15 (0.55-2.42) | 0.91 (0.33-2.50) | 1 |
| Epistaxis | ||||||
| Frequency, n (%) | 8/38 (21.1) | 11/50 (22.0) | 7/66 (10.6) | 3/22 (13.6) | 2/18 (11.1) | 4/32 (12.5) |
| RR (95% CI) | 1.58 (0.91-2.76) | 1.54 (0.99-2.39) | 1 | 1.06 (0.42-2.67) | 0.92 (0.28-3.04) | 1 |
| Hematomas | ||||||
| Frequency, n (%) | 4/38 (10.5) | 2/50 (4.0) | 1/66 (1.5) | 2/22 (9.1) | 0/18 (0.0) | 1/32 (3.1) |
| RR (95% CI) | 2.33 (1.39-3.91) | 1.57 (0.69-3.60) | 1 | 1.70 (0.71-4.06) | — | 1 |
| Bleeding after dental procedures | ||||||
| Frequency, n (%) | 5/38 (13.2) | 2/50 (4.0) | 5/66 (7.6) | 5/22 (22.7) | 0/18 (0.0) | 2/32 (6.3) |
| RR (95% CI) | 1.42 (0.72-2.81) | 0.65 (0.20-2.13) | 1 | 1.98 (1.08-3.61) | — | 1 |
| Perioperative bleeding | ||||||
| Frequency, n (%) | 5/38 (13.2) | 2/50 (4.0) | 8/66 (12.1) | 5/22 (22.7) | 1/18 (5.6) | 2/32 (6.3) |
| RR (95% CI) | 1.06 (0.51-2.22) | 0.44 (0.13-1.55) | 1 | 1.98 (1.08-3.61) | 0.92 (0.18-4.77) | 1 |
| Mucosal bleeding | ||||||
| Frequency, n (%) | 5/38 (13.2) | 10/50 (20.0) | 5/66 (7.6) | 2/22 (9.1) | 1/18 (5.6) | 3/32 (9.4) |
| RR (95% CI) | 1.42 (0.72-2.81) | 1.68 (1.10-2.59) | 1 | 0.98 (0.32-3.02) | 0.68 (0.12-3.85) | 1 |
| Bleeding requiring transfusion | ||||||
| Frequency, n (%) | 0/38 (0.0) | 0/50 (0.0) | 6/66 (9.1) | 3/22 (13.6) | 1/18 (5.6) | 0/32 (0.0) |
| RR (95% CI) | — | — | 1 | 2.68 (1.88-3.83) | 2.88 (1.96-4.23) | 1 |
| Abnormal bleeding after trauma | ||||||
| Frequency, n (%) | 5/38 (13.2) | 2/50 (4.0) | 2/66 (3.0) | 2/22 (9.1) | 0/18 (0.0) | 0/32 (0.0) |
| RR (95% CI) | 2.10 (1.22-3.62) | 1.17 (0.43-3.18) | 1 | 2.60 (1.84-3.67) | — | 1 |
| Hemarthrosis | ||||||
| Frequency, n (%) | 4/38 (10.5) | 0/50 (0.0) | 0/66 (0.0) | 1/22 (4.5) | 0/18 (0.0) | 0/32 (0.0) |
| RR (95% CI) | 2.94 (2.24-3.87) | — | 1 | 2.52 (1.81-3.52) | — | 1 |
| Other bleeding | ||||||
| Frequency, n (%) | 1/38 (2.6) | 0/50 (0.0) | 0/66 (0.0) | 0/22 (0.0) | 0/18 (0.0) | 0/32 (0.0) |
| RR (95% CI) | 2.78 (2.15-3.60) | — | 1 | — | — | 1 |
| Anemia | ||||||
| Frequency, n (%) | 9/38 (23.7) | 3/50 (6.0) | 3/66 (4.5) | 4/22 (18.2) | 4/18 (22.2) | 1/32 (3.1) |
| RR (95% CI) | 2.38 (1.53-3.71) | 1.17 (0.51-2.68) | 1 | 2.18 (1.23-3.86) | 2.57 (1.39-4.77) | 1 |
| Iron deficiency | ||||||
| Frequency, n (%) | 10/38 (26.3) | 4/50 (8.0) | 4/66 (6.1) | 3/22 (13.6) | 2/18 (11.1) | 0/32 (0.0) |
| RR (95% CI) | 2.30 (1.46-3.61) | 1.17 (0.57-2.43) | 1 | 2.68 (1.88-3.83) | 3.00 (2.01-4.48) | 1 |
| . | FVIII level . | FIX level . | ||||
|---|---|---|---|---|---|---|
| <40 IU/dL . | 40-60 IU/dL . | >60 IU/dL . | <40 IU/dL . | 40-60 IU/dL . | >60 IU/dL . | |
| HMB∗ | ||||||
| Frequency, n (%) | 22/35 (62.9) | 20/48 (41.7) | 23/62 (37.1) | 8/18 (44.4) | 5/16 (31.3) | 10/29 (34.5) |
| RR (95% CI) | 1.96 (1.12-3.42) | 1.11 (0.73-1.71) | 1 | 1.29 (0.63-2.65) | 0.91 (0.39-2.14) | 1 |
| RTB∗,† | ||||||
| Frequency, n (%) | 2/35 (5.7) | 5/48 (10.4) | 7/62 (11.3) | 7/18 (38.9) | 2/16 (12.5) | 3/29 (10.3) |
| RR (95% CI) | 0.59 (0.17-2.07) | 0.95 (0.47-1.92) | 1 | 2.36 (1.24-4.47) | 1.14 (0.36-3.62) | 1 |
| Easy bruising | ||||||
| Frequency, n (%) | 16/38 (42.1) | 20/50 (40.0) | 17/66 (25.8) | 5/22 (22.7) | 3/18 (16.7) | 6/32 (18.8) |
| RR (95% CI) | 1.57 (0.96-2.57) | 1.42 (0.95-2.14) | 1 | 1.15 (0.55-2.42) | 0.91 (0.33-2.50) | 1 |
| Epistaxis | ||||||
| Frequency, n (%) | 8/38 (21.1) | 11/50 (22.0) | 7/66 (10.6) | 3/22 (13.6) | 2/18 (11.1) | 4/32 (12.5) |
| RR (95% CI) | 1.58 (0.91-2.76) | 1.54 (0.99-2.39) | 1 | 1.06 (0.42-2.67) | 0.92 (0.28-3.04) | 1 |
| Hematomas | ||||||
| Frequency, n (%) | 4/38 (10.5) | 2/50 (4.0) | 1/66 (1.5) | 2/22 (9.1) | 0/18 (0.0) | 1/32 (3.1) |
| RR (95% CI) | 2.33 (1.39-3.91) | 1.57 (0.69-3.60) | 1 | 1.70 (0.71-4.06) | — | 1 |
| Bleeding after dental procedures | ||||||
| Frequency, n (%) | 5/38 (13.2) | 2/50 (4.0) | 5/66 (7.6) | 5/22 (22.7) | 0/18 (0.0) | 2/32 (6.3) |
| RR (95% CI) | 1.42 (0.72-2.81) | 0.65 (0.20-2.13) | 1 | 1.98 (1.08-3.61) | — | 1 |
| Perioperative bleeding | ||||||
| Frequency, n (%) | 5/38 (13.2) | 2/50 (4.0) | 8/66 (12.1) | 5/22 (22.7) | 1/18 (5.6) | 2/32 (6.3) |
| RR (95% CI) | 1.06 (0.51-2.22) | 0.44 (0.13-1.55) | 1 | 1.98 (1.08-3.61) | 0.92 (0.18-4.77) | 1 |
| Mucosal bleeding | ||||||
| Frequency, n (%) | 5/38 (13.2) | 10/50 (20.0) | 5/66 (7.6) | 2/22 (9.1) | 1/18 (5.6) | 3/32 (9.4) |
| RR (95% CI) | 1.42 (0.72-2.81) | 1.68 (1.10-2.59) | 1 | 0.98 (0.32-3.02) | 0.68 (0.12-3.85) | 1 |
| Bleeding requiring transfusion | ||||||
| Frequency, n (%) | 0/38 (0.0) | 0/50 (0.0) | 6/66 (9.1) | 3/22 (13.6) | 1/18 (5.6) | 0/32 (0.0) |
| RR (95% CI) | — | — | 1 | 2.68 (1.88-3.83) | 2.88 (1.96-4.23) | 1 |
| Abnormal bleeding after trauma | ||||||
| Frequency, n (%) | 5/38 (13.2) | 2/50 (4.0) | 2/66 (3.0) | 2/22 (9.1) | 0/18 (0.0) | 0/32 (0.0) |
| RR (95% CI) | 2.10 (1.22-3.62) | 1.17 (0.43-3.18) | 1 | 2.60 (1.84-3.67) | — | 1 |
| Hemarthrosis | ||||||
| Frequency, n (%) | 4/38 (10.5) | 0/50 (0.0) | 0/66 (0.0) | 1/22 (4.5) | 0/18 (0.0) | 0/32 (0.0) |
| RR (95% CI) | 2.94 (2.24-3.87) | — | 1 | 2.52 (1.81-3.52) | — | 1 |
| Other bleeding | ||||||
| Frequency, n (%) | 1/38 (2.6) | 0/50 (0.0) | 0/66 (0.0) | 0/22 (0.0) | 0/18 (0.0) | 0/32 (0.0) |
| RR (95% CI) | 2.78 (2.15-3.60) | — | 1 | — | — | 1 |
| Anemia | ||||||
| Frequency, n (%) | 9/38 (23.7) | 3/50 (6.0) | 3/66 (4.5) | 4/22 (18.2) | 4/18 (22.2) | 1/32 (3.1) |
| RR (95% CI) | 2.38 (1.53-3.71) | 1.17 (0.51-2.68) | 1 | 2.18 (1.23-3.86) | 2.57 (1.39-4.77) | 1 |
| Iron deficiency | ||||||
| Frequency, n (%) | 10/38 (26.3) | 4/50 (8.0) | 4/66 (6.1) | 3/22 (13.6) | 2/18 (11.1) | 0/32 (0.0) |
| RR (95% CI) | 2.30 (1.46-3.61) | 1.17 (0.57-2.43) | 1 | 2.68 (1.88-3.83) | 3.00 (2.01-4.48) | 1 |