Table 3.
Baseline characteristics and efficacy outcomes by inhibitor boosting
|
Inhibitor boosting (n= 9) |
No inhibitor boosting (n= 10) |
|
|---|---|---|
| Gender, n (%) | ||
| Male | 3 (33.3) | 6 (60) |
| Female | 6 (66.7) | 4 (40) |
| Age (years), median (range) | 50.0 (23–84) | 66.5 (22–86) |
| Previous TTP episode, n (%) | ||
| No | 9 (100) | 5 (50) |
| Yes | 0 | 5 (50) |
| Disease severity at baseline, n (%) | ||
| Very severe | 6 (66.7) | 5 (50) |
| Less severe | 3 (33.3) | 5 (50) |
| Platelet count (109/L) at baseline, median (range) | 12.0 (8–65) | 31.0 (9–78) |
| Serum creatine (µmol/L) at baseline, median (range) | 67.0 (64–226) | 87.0 (49–162) |
| Lactate dehydrogenase (U/L) at baseline, median (range) | 575.0 (227–1794) | 452.5 (234–947) |
| Cardiac troponin level (µg/L) at baseline, median (range) | 0.310 (0.03– 3.52) | 0.055 (0.03–7.47) |
| ADAMTS13 inhibitor (BU/mL) at baseline, median (range) | 1.80 (1.5–8.3) | 1.45 (0.5–6.1) |
| Time to platelet count response (days), median (95% CI) | 2.42 (0.88–3.59) | 3.98 (1.69–NC) |
| Time to normalization of all 3 organ damage marker levels (days), median (95% CI) | 4.48 (0.87–11.40) | 3.20 (0.79–4.98) |
| Number of days of TPE, median (range) | 5.0 (3–11) | 6.5 (5–20) |
| Use of concomitant rituximab, n (%) | 8 (88.9) | 5 (50.0) |
| Before completion of TPE | 0 | 4 (40.0) |
| Day of TPE completion | 1 (11.1)a | 0 |
| After completion of TPE | 7 (77.8) | 1 (10.0) |
| Time to normalization of serum creatine (days), median (95% CI) | 1.83 (0.49–NC) | 1.92 (0.98–NC) |
| Time to normalization of cardiac troponin I level (days), median (95% CI) | 6.31 ((1.49–NC) | 3.06 (1.92–NC) |
| Time to normalization of lactate dehydrogenase (days), median (95% CI) | 2.54 (0.87–NC) | 0.98 (0.46–4.98) |
CI Confidence interval, iTTP Immune-mediated thrombotic thrombocytopenic purpura, NC Not calculated, TPE Therapeutic plasma exchange, TTP Thrombotic thrombocytopenic purpura
aOne patient experienced a recurrence, 2 days after discontinuing caplacizumab due to an adverse event