Comparison of clinical characteristics of hemophilia B patients with only SP domain deletions and deletions beyond SP domain
| Variables . | Deletion beyond SP . | SP deletion only . | P value . |
|---|---|---|---|
| All patients, N | 18 | 10 | – |
| Inhibitor development, n (%) | 17 (94.4) | 2 (20.0) | <.001 |
| Allergic reaction, n (%) | 12 (66.7) | 1 (10.0) | .006 |
| Information on inhibitor development, N | 17 | 2 | – |
| Age at inhibitor diagnosis, y, M (range) | 4.5 (1.2-12.6) | (1.6, 13.4)∗ | – |
| Eds of inhibitor development, d, M (range) | 30 (5-200) | (18, 200)∗ | – |
| Titer at inhibitor diagnosis, BU/mL, M (range) | 14 (0.7-160.0) | (1.9, 6.2)∗ | – |
| Historical peak inhibitor titer, BU/mL, M (range) | 60.0 (1.6-500.0) | (6.8, 7.7)∗ | – |
| Patients undergoing ITI treatment, N | 11 | 2 | – |
| Pre-ITI inhibitor titer, BU/mL, M (range) | 16.8 (2.0-256.0) | (5.0, 6.8)∗ | – |
| Age at ITI start, y, mean ± SD | 8.6 ± 4.7 | (1.7, 13.6)∗ | – |
| The interval from inhibitor diagnosis to ITI start, mo, mean ± SD | 26.9 ± 23.5 | (0.9, 2.8)∗ | – |
| Peak inhibitor titer during ITI, BU/mL, M (range) | 30.4 (2.7-363.5) | (0.8, 3.2)∗ | – |
| Treatment regimen of ITI-IS, n (%) | 11 (100.0) | 2 (100.0) | – |
| ITI success, n (%) | 5 (45.5) | 2 (100.0) | – |
| Time to success, mo, M (range) | 1.94 (1.4-43.3) | (0.9, 1.8)∗ | – |
| Relapse, n (%) | 3 (60.0) | 0 (0.0) | – |
| Nephrotic syndrome, n (%) | 2 (18.2) | 0 (0) | – |
| Variables . | Deletion beyond SP . | SP deletion only . | P value . |
|---|---|---|---|
| All patients, N | 18 | 10 | – |
| Inhibitor development, n (%) | 17 (94.4) | 2 (20.0) | <.001 |
| Allergic reaction, n (%) | 12 (66.7) | 1 (10.0) | .006 |
| Information on inhibitor development, N | 17 | 2 | – |
| Age at inhibitor diagnosis, y, M (range) | 4.5 (1.2-12.6) | (1.6, 13.4)∗ | – |
| Eds of inhibitor development, d, M (range) | 30 (5-200) | (18, 200)∗ | – |
| Titer at inhibitor diagnosis, BU/mL, M (range) | 14 (0.7-160.0) | (1.9, 6.2)∗ | – |
| Historical peak inhibitor titer, BU/mL, M (range) | 60.0 (1.6-500.0) | (6.8, 7.7)∗ | – |
| Patients undergoing ITI treatment, N | 11 | 2 | – |
| Pre-ITI inhibitor titer, BU/mL, M (range) | 16.8 (2.0-256.0) | (5.0, 6.8)∗ | – |
| Age at ITI start, y, mean ± SD | 8.6 ± 4.7 | (1.7, 13.6)∗ | – |
| The interval from inhibitor diagnosis to ITI start, mo, mean ± SD | 26.9 ± 23.5 | (0.9, 2.8)∗ | – |
| Peak inhibitor titer during ITI, BU/mL, M (range) | 30.4 (2.7-363.5) | (0.8, 3.2)∗ | – |
| Treatment regimen of ITI-IS, n (%) | 11 (100.0) | 2 (100.0) | – |
| ITI success, n (%) | 5 (45.5) | 2 (100.0) | – |
| Time to success, mo, M (range) | 1.94 (1.4-43.3) | (0.9, 1.8)∗ | – |
| Relapse, n (%) | 3 (60.0) | 0 (0.0) | – |
| Nephrotic syndrome, n (%) | 2 (18.2) | 0 (0) | – |
BU, Bethesda unit; Eds, exposure days; IS, immunosuppressant; M, median; SD, standard deviation.
The values in parentheses were the values of the corresponding variables for patients 15 or 16, respectively.