Hospitalizations and FVIII treatment regimens for HIPS subjects, stratified in 4 subgroups with distinct signatures of FVIII-binding antibodies developed during the HIPS study
| Patient no. . | Hospitalizations during study . | EDs when prophylaxis and ITI (if applicable) were initiated (FVIII dose in IU/kg and treatment frequency) . |
|---|---|---|
| Subgroup 1: no FVIII-binding IgG antibodies | ||
| 1 | No hospitalizations reported | ED1 (55 qw, 55 biw, 75 biw) |
| 2 | 4 hospitalizations: tongue bleed, head trauma, muscle bleed, port implant | ED35 (25 qw, 25 biw) |
| 3 | 1 hospitalization: port implant and circumcision | ED13 (25 biw & 50 qw; 50 biw & 70 qw) |
| 4 | No hospitalizations reported | ED5 (50 biw, 45 biw) |
| 5 | No hospitalizations reported | ED5 (90 qw, 55qw, 65 qw, 60 biw) |
| 6 | No hospitalizations reported | ED1 (70 qw, 95 qw, 60 qw, 60 biw) |
| 7 | No hospitalizations reported | ED1 (70 qw, 45 qw, 20 biw, 20 tiw) |
| Subgroup 2: nonneutralizing FVIII-binding antibodies | ||
| 8 | 1 hospitalization: port implant | ED1 (25 biw, 25 tiw) |
| 9 | No hospitalizations reported | ED3 (55 qw, 55 biw) |
| 10 | 3 hospitalizations: muscle bleed, left knee joint bleed, port implant | ED7 (25 – 55 qw, 25 biw, 20 tiw) |
| 11 | No hospitalizations reported | ED17 (25 tiw) |
| 12 | 1 hospitalization: muscle bleed (back) | ED1 (50 qw, 60 qw, 35 biw) |
| 13 | No hospitalizations reported | ED6 (30 qw, 30 biw) |
| 14 | No hospitalizations reported | ED8 (40 qw, 50 qw, 50 biw) |
| Subgroup 3: transient FVIII inhibitors | ||
| 15 | No hospitalizations reported | ED6 (60 qw, 45 qw, 50 qw) |
| 16 | 1 hospitalization: port implant (procedure occurred 3 mo after first inhibitor detection) | ED3 (90 qw, 55 qw, 65 biw) |
| Subgroup 4: persistent FVIII inhibitors | ||
| 17 | 1 hospitalization: port implant (procedure occurred 20 d prior to inhibitor detection) | ED1 (55 qw); ITI 225 qd (initiated on ED12) |
| 18 | No hospitalizations reported | ED1 (45 - 60 qw); ITI 210 qd (initiated on ED7) |
| 19 | 2 hospitalizations: intraspinal bleed and port implant, MRI and diagnosis after intraspinal bleed (all events occurred 1.5 y after inhibitor detection) | ED2 (35 qw); ITI 105 qd (initiated on ED13) |
| 20 | 1 hospitalization: port implant (procedure occurred 2 wk after inhibitor detection) | ED2 (120 qw); ITI 560 qd (initiated on ED16) |
| 21 | 3 hospitalizations: port implant (3 d prior to inhibitor detection), 2 infections (1 wk after inhibitor detection) | ED1 (30 qw); ITI 190 qd (initiated on ED11) |
| 22 | 3 hospitalizations: 2 fistulas, 1 hematoma episode (all events occurred after inhibitor detection) | ED1: 35 biw, ITI 210 qd (initiated on ED12) |
| 23 | 1 hospitalization: shunt for venous access (procedure occurred 2 mo after inhibitor detection) | ED3: 30 qw, ITI 155 qd (initiated on ED13) |
| Patient no. . | Hospitalizations during study . | EDs when prophylaxis and ITI (if applicable) were initiated (FVIII dose in IU/kg and treatment frequency) . |
|---|---|---|
| Subgroup 1: no FVIII-binding IgG antibodies | ||
| 1 | No hospitalizations reported | ED1 (55 qw, 55 biw, 75 biw) |
| 2 | 4 hospitalizations: tongue bleed, head trauma, muscle bleed, port implant | ED35 (25 qw, 25 biw) |
| 3 | 1 hospitalization: port implant and circumcision | ED13 (25 biw & 50 qw; 50 biw & 70 qw) |
| 4 | No hospitalizations reported | ED5 (50 biw, 45 biw) |
| 5 | No hospitalizations reported | ED5 (90 qw, 55qw, 65 qw, 60 biw) |
| 6 | No hospitalizations reported | ED1 (70 qw, 95 qw, 60 qw, 60 biw) |
| 7 | No hospitalizations reported | ED1 (70 qw, 45 qw, 20 biw, 20 tiw) |
| Subgroup 2: nonneutralizing FVIII-binding antibodies | ||
| 8 | 1 hospitalization: port implant | ED1 (25 biw, 25 tiw) |
| 9 | No hospitalizations reported | ED3 (55 qw, 55 biw) |
| 10 | 3 hospitalizations: muscle bleed, left knee joint bleed, port implant | ED7 (25 – 55 qw, 25 biw, 20 tiw) |
| 11 | No hospitalizations reported | ED17 (25 tiw) |
| 12 | 1 hospitalization: muscle bleed (back) | ED1 (50 qw, 60 qw, 35 biw) |
| 13 | No hospitalizations reported | ED6 (30 qw, 30 biw) |
| 14 | No hospitalizations reported | ED8 (40 qw, 50 qw, 50 biw) |
| Subgroup 3: transient FVIII inhibitors | ||
| 15 | No hospitalizations reported | ED6 (60 qw, 45 qw, 50 qw) |
| 16 | 1 hospitalization: port implant (procedure occurred 3 mo after first inhibitor detection) | ED3 (90 qw, 55 qw, 65 biw) |
| Subgroup 4: persistent FVIII inhibitors | ||
| 17 | 1 hospitalization: port implant (procedure occurred 20 d prior to inhibitor detection) | ED1 (55 qw); ITI 225 qd (initiated on ED12) |
| 18 | No hospitalizations reported | ED1 (45 - 60 qw); ITI 210 qd (initiated on ED7) |
| 19 | 2 hospitalizations: intraspinal bleed and port implant, MRI and diagnosis after intraspinal bleed (all events occurred 1.5 y after inhibitor detection) | ED2 (35 qw); ITI 105 qd (initiated on ED13) |
| 20 | 1 hospitalization: port implant (procedure occurred 2 wk after inhibitor detection) | ED2 (120 qw); ITI 560 qd (initiated on ED16) |
| 21 | 3 hospitalizations: port implant (3 d prior to inhibitor detection), 2 infections (1 wk after inhibitor detection) | ED1 (30 qw); ITI 190 qd (initiated on ED11) |
| 22 | 3 hospitalizations: 2 fistulas, 1 hematoma episode (all events occurred after inhibitor detection) | ED1: 35 biw, ITI 210 qd (initiated on ED12) |
| 23 | 1 hospitalization: shunt for venous access (procedure occurred 2 mo after inhibitor detection) | ED3: 30 qw, ITI 155 qd (initiated on ED13) |
The last column, “EDs when prophylaxis and ITI (if applicable) were initiated,” includes the dose and frequency of prophylaxis and any changes to dose and frequency. Dose was calculated using the most recent prior weight measured (at screening or interval visit) and the FVIII dose in IU/kg reported on the infusion diary. All subjects received prophylaxis at some stage during the HIPS study. Subjects who did not start prophylaxis on ED1 were treated on demand until the exposure day specified in the table.
biw, twice a week; ITI, immune tolerance induction; MRI, magnetic resonance imaging; qd, daily; qw, once a week; tiw, 3 times a week.