Treatment type and frequency by age
| Age, y . | Prophylactic FFP . | Prophylactic BPL 8Y . | On demand . | No treatment . |
|---|---|---|---|---|
| <16 (n = 17) | n = 0 | n = 16 | n = 1 | n = 0 |
| Once/wk, n = 5 | ||||
| Every 10 d, n = 1 | ||||
| Every 2 wk, n = 6 | ||||
| Every 3 wk, n = 2 | ||||
| Every 4 wk, n = 2 | ||||
| ≥16 (n = 56) | n = 23 | n = 10 | n = 8 | n = 15 |
| Three/wk, n = 1 | Twice/wk, n = 6 | |||
| Once/wk, n = 5 | Once/wk, n = 4 | |||
| Every 2 wk, n = 11 | ||||
| Every 3 wk, n = 6 | ||||
| Every month, n = 1 |
| Age, y . | Prophylactic FFP . | Prophylactic BPL 8Y . | On demand . | No treatment . |
|---|---|---|---|---|
| <16 (n = 17) | n = 0 | n = 16 | n = 1 | n = 0 |
| Once/wk, n = 5 | ||||
| Every 10 d, n = 1 | ||||
| Every 2 wk, n = 6 | ||||
| Every 3 wk, n = 2 | ||||
| Every 4 wk, n = 2 | ||||
| ≥16 (n = 56) | n = 23 | n = 10 | n = 8 | n = 15 |
| Three/wk, n = 1 | Twice/wk, n = 6 | |||
| Once/wk, n = 5 | Once/wk, n = 4 | |||
| Every 2 wk, n = 11 | ||||
| Every 3 wk, n = 6 | ||||
| Every month, n = 1 |
Patients either received regular prophylactic FFP or BPL 8Y or no regular prophylactic treatment. For patients not on prophylaxis, on-demand treatment was given during events precipitating a cTTP exacerbation such as pregnancy or infection. The on-demand column signifies patients who required on-demand treatment and the no-treatment column patients who had never needed or received treatment since their initial presenting event. The child who needed on-demand therapy received BPL 8Y whereas all 8 adults who needed on-demand treatment were given FFP.