HLH-related therapies used before and concurrently with emapalumab treatment among patients with pHLH
| Parameter . | Overall (N = 46) . | Children (aged <12 y) (n = 39)∗ . | Adolescents and adults (aged ≥12 y) (n = 7)∗ . |
|---|---|---|---|
| Therapies before emapalumab treatment | |||
| Patients who received other HLH therapies, n (%) | 35 (76.1) | 30 (76.9) | 5 (71.4) |
| Corticosteroids | 35 (100.0) | 30 (100.0) | 5 (100.0) |
| Etoposide | 20 (57.1) | 18 (60.0) | 2 (40.0) |
| Anakinra | 4 (11.4) | 3 (10.0) | 1 (20.0) |
| Methotrexate + hydrocortisone | 2 (5.7) | 1 (3.3) | 1 (20.0) |
| Cyclosporine | 1 (2.9) | 1 (3.3) | 0 |
| Alemtuzumab | 1 (2.9) | 1 (3.3) | 0 |
| Rituximab | 1 (2.9) | 0 | 1 (20.0) |
| Basiliximab | 1 (2.9) | 1 (3.3) | 0 |
| Therapies concurrent with emapalumab treatment | |||
| Patients who received other HLH-related therapies, n (%) | 44/46 (95.7) | 38/39 (97.4) | 6/7 (85.7) |
| Corticosteroids | 44/44 (100.0) | 38/38 (100.0) | 6/6 (100.0) |
| Etoposide | 22/44 (50.0) | 20/38 (52.6) | 2/6 (33.3) |
| Anakinra | 6/44 (13.6) | 6/38 (15.8) | 0 |
| Methotrexate + hydrocortisone | 5/44 (11.4) | 4/38 (10.5) | 1/6 (16.7) |
| Ruxolitinib | 4/44 (9.1) | 4/38 (10.5) | 0 |
| Alemtuzumab | 3/44 (6.8) | 3/38 (7.9) | 0 |
| Cyclophosphamide | 3/44 (6.8) | 1/38 (2.6) | 2/6 (33.3) |
| Cyclosporine | 3/44 (6.8) | 3/38 (7.9) | 0 |
| Rituximab | 2/44 (4.5) | 1/38 (2.6) | 1/6 (16.7) |
| Doxorubicin | 1/44 (2.3) | 0 | 1/6 (16.7) |
| Tocilizumab | 1/44 (2.3) | 1/38 (2.6) | 0 |
| Nivolumab | 1/44 (2.3) | 1/38 (2.6) | 0 |
| Abatacept | 1/44 (2.3) | 1/38 (2.6) | 0 |
| Parameter . | Overall (N = 46) . | Children (aged <12 y) (n = 39)∗ . | Adolescents and adults (aged ≥12 y) (n = 7)∗ . |
|---|---|---|---|
| Therapies before emapalumab treatment | |||
| Patients who received other HLH therapies, n (%) | 35 (76.1) | 30 (76.9) | 5 (71.4) |
| Corticosteroids | 35 (100.0) | 30 (100.0) | 5 (100.0) |
| Etoposide | 20 (57.1) | 18 (60.0) | 2 (40.0) |
| Anakinra | 4 (11.4) | 3 (10.0) | 1 (20.0) |
| Methotrexate + hydrocortisone | 2 (5.7) | 1 (3.3) | 1 (20.0) |
| Cyclosporine | 1 (2.9) | 1 (3.3) | 0 |
| Alemtuzumab | 1 (2.9) | 1 (3.3) | 0 |
| Rituximab | 1 (2.9) | 0 | 1 (20.0) |
| Basiliximab | 1 (2.9) | 1 (3.3) | 0 |
| Therapies concurrent with emapalumab treatment | |||
| Patients who received other HLH-related therapies, n (%) | 44/46 (95.7) | 38/39 (97.4) | 6/7 (85.7) |
| Corticosteroids | 44/44 (100.0) | 38/38 (100.0) | 6/6 (100.0) |
| Etoposide | 22/44 (50.0) | 20/38 (52.6) | 2/6 (33.3) |
| Anakinra | 6/44 (13.6) | 6/38 (15.8) | 0 |
| Methotrexate + hydrocortisone | 5/44 (11.4) | 4/38 (10.5) | 1/6 (16.7) |
| Ruxolitinib | 4/44 (9.1) | 4/38 (10.5) | 0 |
| Alemtuzumab | 3/44 (6.8) | 3/38 (7.9) | 0 |
| Cyclophosphamide | 3/44 (6.8) | 1/38 (2.6) | 2/6 (33.3) |
| Cyclosporine | 3/44 (6.8) | 3/38 (7.9) | 0 |
| Rituximab | 2/44 (4.5) | 1/38 (2.6) | 1/6 (16.7) |
| Doxorubicin | 1/44 (2.3) | 0 | 1/6 (16.7) |
| Tocilizumab | 1/44 (2.3) | 1/38 (2.6) | 0 |
| Nivolumab | 1/44 (2.3) | 1/38 (2.6) | 0 |
| Abatacept | 1/44 (2.3) | 1/38 (2.6) | 0 |
Patients were classified as children (aged <12 years) and adolescents and adults (aged ≥12 years) based on age at time of emapalumab initiation.