Parameter Hemophilia A Hemophilia B
Moderate
(n=66)
Severe
(n=115)
Moderate and
Severe (n=181)
Moderate
(n=28)
Severe
(n=79)
Moderate and
Severe (n=107)
Bleeding outcomes
ABR, mean (SD) 2.77 (3.57) 4.36 (9.38) 3.78 (7.80) 3.64 (4.27) 4.57 (6.44) 4.33 (5.94)
Cause of bleeding (proportion), % (SD)
Trauma-related bleeding events 59.14 (29.43) 58.70 (29.52) 58.86 (29.41) 67.86 (29.48) 60.03 (27.93) 62.11 (28.42)
Spontaneous bleeding events 40.86 (29.43) 41.30 (29.52) 41.14 (29.41) 32.14 (29.48) 39.97 (27.93) 37.89 (28.42)
Target joints, n (%)a 23 (34.85) 61 (53.04) 84 (46.41) 9 (32.14) 20 (25.32) 29 (27.10)
Distribution of target joints, n (%)
Ankle 3 (4.55) 12 (10.43) 15 (8.29) 2 (7.14) 4 (5.06) 6 (5.61)
Elbow 2 (3.03) 12 (10.43) 14 (7.73) 3 (10.71) 5 (6.33) 8 (7.48)
Hip 6 (9.09) 13 (11.30) 19 (10.50) 1 (3.57) 7 (8.86) 8 (7.48)
Knee 14 (21.21) 44 (38.26) 58 (32.04) 3 (10.71) 9 (11.39) 12 (11.21)
Neck 1 (1.52) 1 (0.87) 2 (1.10) 2 (7.14) 1 (1.27) 3 (2.80)
Shoulder 4 (6.06) 7 (6.09) 11 (6.08) 3 (10.71) 2 (2.53) 5 (4.67)
Spine 1 (1.52) 1 (0.87) 2 (1.10) NR 2 (2.53) 2 (1.87)
Wrist 1 (1.52) 6 (5.22) 7 (3.87) 1 (3.57) NR 1 (0.93)
Problem joints, n (%) b 31 (46.97) 55 (47.83) 86 (47.51) 8 (28.57) 38 (48.10) 46 (42.99)
Distribution of problem joints, n (%)
Ankle 12 (18.18) 16 (13.91) 28 (15.47) 1 (3.57) 12 (15.19) 13 (12.15)
Elbow 2 (3.03) 9 (7.83) 11 (6.08) 0 (0.00) 5 (6.33) 5 (4.67)
Hip 9 (13.64) 10 (8.70) 19 (10.50) NR 7 (8.86) 7 (6.54)
Knee 17 (25.76) 31 (26.96) 48 (26.52) 3 (10.71) 22 (27.85) 25 (23.36)
Neck NR NR NR NR NR NR
Shoulder 2 (3.03) 9 (7.83) 11 (6.08) 2 (7.14) 4 (5.06) 6 (5.61)
Spine 4 (6.06) 3 (2.61) 7 (3.87) 1 (3.57) NR 1 (0.93)
Wrist 4 (6.06) 6 (5.22) 10 (5.52) 1 (3.57) 4 (5.06) 5 (4.67)
Joint procedures (in prior 12 months),
n (%)
10 (15.15) 29 (25.22) 39 (21.55) 1 (3.57) 5 (6.33) 6 (5.61)
Distribution of joint procedures, n (%)
Ankle 2 (3.03) NR 2 (1.10) NR 1 (1.27) 1 (0.93)
Elbow NR 1 (0.87) 1 (0.55) NR 1 (1.27) 1 (0.93)
Hip 1 (1.52) 5 (4.35) 6 (3.31) NR 1 (1.27) 1 (0.93)
Knee 6 (9.09) 26 (22.61) 32 (17.68) NR 1 (1.27) 1 (0.93)
Neck NR NR NR NR 1 (1.27) 1 (0.93)
Shoulder 2 (3.03) 3 (2.61) 5 (2.76) 1 (3.57) NR 1 (0.93)
Spine NR NR NR NR NR NR
Wrist 1 (1.52) NR 1 (0.55) NR NR NR
Surgical intervention type, n (%)
Arthrocentesis 9 (13.64) 24 (20.87) 33 (18.23) NR 6 (7.59) 6 (5.61)
Arthroscopy 6 (9.09) 9 (7.83) 15 (8.29) NR 3 (3.80) 3 (2.80)
Arthrodesis 2 (3.03) 5 (4.35) 7 (3.87) NR NR NR
Synovectomy 1 (1.52) 3 (2.61) 4 (2.21) 1 (3.57) 2 (2.53) 3 (2.80)
Arthroplasty 2 (3.03) 2 (1.74) 4 (2.21) NR 1 (1.27) 1 (0.93)
No. of joint procedures, mean (SD; n)
Total sample 0.58 (2.17; 66) 0.63 (1.76; 115) 0.61 (1.91; 181) 0.04 (0.19; 28) 0.15 (0.46; 79) 0.12 (0.41; 107)
Patients with ≥1 procedure 3.80 (4.52; 10) 2.48 (2.80; 29) 2.82 (3.31; 39) 1.00 (NA; 1) 1.33 (0.48; 9) 1.30 (0.48; 10)
No. of hospital admissions, mean (SD; n)
Related to bleeding events c
Occurrences in the total sample 0.61 (0.70; 66) 0.72 (1.03; 115) 0.68 (0.92; 181) 0.50 (0.81; 28) 1.11 (0.81; 79) 0.89 (0.86; 107)
Occurrences in patients with ≥1 bleed-related hospitalization 1.21 (0.48; 33) 1.73 (0.89; 48) 1.52 (0.79; 81) 1.44 (0.73; 9) 1.36 (0.68; 36) 1.38 (0.68; 45)
Related to joint procedures d
Occurrences in the total sample 0.36 (1.35; 66) 0.39 (1.01; 115) 0.38 (1.14; 181) 0.04 (0.19; 28) 0.14 (0.42; 79) 0.11 (0.37; 107)
Occurrences in patients with ≥1 procedure-related ward stays 2.67 (2.83; 9) 1.88 (1.45; 24) 2.09 (1.91; 33) 1.00 (NA; 1) 1.22 (0.44; 9) 1.20 (0.42; 10)
Chronic pain level related to hemophilia, n (%)
No pain 18 (27.27) 33 (28.70) 51 (28.18) 10 (35.71) 8 (10.13) 18 (16.82)
Mild pain 24 (36.36) 34 (29.57) 58 (32.04) 11 (39.29) 40 (50.63) 51 (47.66)
Moderate pain 22 (33.33) 38 (33.04) 60 (33.15) 7 (25.00) 26 (32.91) 33 (30.84)
Severe pain 2 (3.03) 10 (8.70) 12 (6.63) NR 5 (6.33) 5 (4.67)