Characteristics at presentation of patients receiving caplacizumab
| Baseline characteristics . | Caplacizumab cohort (n = 85) . |
|---|---|
| Age, mean (range), y | 46 (3-82) |
| Females | 56 (66) |
| Ethnicity* | |
| White | 57 (67) |
| Black | 16 (19) |
| Asian | 6 (7) |
| Other | 4 (5) |
| Platelet count, median (IQR), ×109/L | 13 (9-21) |
| Cardiac troponin, median (IQR), ng/mL† | 98 (31-317) |
| Serum creatinine, median (IQR), μmol/L | 90 (71-135) |
| Neurological symptoms at presentation | 56 (66) |
| ADAMTS13 activity | |
| <10 IU/dL | 84 (99) |
| ≥10 IU/dL | 1 (1) |
| Detectable ADAMTS13 inhibitor, U/mL‡ | 68 (80) |
| Immunosuppressive/adjuvant therapy started during admission | |
| Glucocorticoids | 84 (99) |
| Rituximab | 84 (99) |
| Mycophenolate mofetil | 25 (29) |
| Bortezomib | 5 (6) |
| Ofatumumab | 2 (2) |
| Cyclophosphamide | 1 (1) |
| N-acetyl cysteine | 1 (1) |
| Intensive care intervention | |
| Patients requiring intubation | 22 (26) |
| Duration of intubation, median (IQR), d | 4 (2-7) |
| Baseline characteristics . | Caplacizumab cohort (n = 85) . |
|---|---|
| Age, mean (range), y | 46 (3-82) |
| Females | 56 (66) |
| Ethnicity* | |
| White | 57 (67) |
| Black | 16 (19) |
| Asian | 6 (7) |
| Other | 4 (5) |
| Platelet count, median (IQR), ×109/L | 13 (9-21) |
| Cardiac troponin, median (IQR), ng/mL† | 98 (31-317) |
| Serum creatinine, median (IQR), μmol/L | 90 (71-135) |
| Neurological symptoms at presentation | 56 (66) |
| ADAMTS13 activity | |
| <10 IU/dL | 84 (99) |
| ≥10 IU/dL | 1 (1) |
| Detectable ADAMTS13 inhibitor, U/mL‡ | 68 (80) |
| Immunosuppressive/adjuvant therapy started during admission | |
| Glucocorticoids | 84 (99) |
| Rituximab | 84 (99) |
| Mycophenolate mofetil | 25 (29) |
| Bortezomib | 5 (6) |
| Ofatumumab | 2 (2) |
| Cyclophosphamide | 1 (1) |
| N-acetyl cysteine | 1 (1) |
| Intensive care intervention | |
| Patients requiring intubation | 22 (26) |
| Duration of intubation, median (IQR), d | 4 (2-7) |
Unless otherwise noted, data are n (%).
Ethnicity was determined by the hematologist submitting the data for each center. Data on race were missing for 2 patients.
Median cardiac troponin on admission is based on n = 81 patients for whom data were available. Data are based on troponin T, the more commonly used cardiac enzyme in the NHS Trusts submitting the data.
ADAMTS13 inhibitor value on admission is based on n = 78 patients for whom data were available. The NHS Trusts included in this study used different assays for ADAMTS13 inhibitor; therefore, the normal values for each laboratory were used to determine whether the ADAMTS13 inhibitor was present/absent.