First-line treatments for adults with LCH
| N = 196 . | U . | SS-Pulm . | MS/MF RO− . | RO+ . | Total, n (%) . |
|---|---|---|---|---|---|
| Surgical resection or excision | 43 | 1 | 14 | 2 | 60 (30.6) |
| Smoking cessation or reduction | 2 | 42 | 12 | 2 | 58 (29.6) |
| Systemic cytotoxic therapy | 4 | 5 | 20 | 12 | 41 (20.9) |
| Cladribine based | 2 | 2 | 12 | 10 | 26 (13.3) |
| Vinblastine-/vincristine-based with steroid | 0 | 0 | 5 | 2 | 7 (3.6) |
| Cytarabine based | 0 | 1 | 3 | 0 | 4 (2) |
| Others | 2 | 2 | 1 | 0 | 5 (2.6) |
| Systemic corticosteroids | 1 | 16 | 8 | 6 | 31 (15.8) |
| Radiation therapy | 8 | 0 | 10 | 1 | 19 (9.7) |
| Topical immunosuppression | 2 | 0 | 4 | 0 | 6 (3.1) |
| Targeted therapy∗ | 1 | 1 | 2 | 0 | 4 (2) |
| Immune suppression with antiresorptive therapy | 0 | 0 | 1 | 0 | 1 (0.5) |
| N = 196 . | U . | SS-Pulm . | MS/MF RO− . | RO+ . | Total, n (%) . |
|---|---|---|---|---|---|
| Surgical resection or excision | 43 | 1 | 14 | 2 | 60 (30.6) |
| Smoking cessation or reduction | 2 | 42 | 12 | 2 | 58 (29.6) |
| Systemic cytotoxic therapy | 4 | 5 | 20 | 12 | 41 (20.9) |
| Cladribine based | 2 | 2 | 12 | 10 | 26 (13.3) |
| Vinblastine-/vincristine-based with steroid | 0 | 0 | 5 | 2 | 7 (3.6) |
| Cytarabine based | 0 | 1 | 3 | 0 | 4 (2) |
| Others | 2 | 2 | 1 | 0 | 5 (2.6) |
| Systemic corticosteroids | 1 | 16 | 8 | 6 | 31 (15.8) |
| Radiation therapy | 8 | 0 | 10 | 1 | 19 (9.7) |
| Topical immunosuppression | 2 | 0 | 4 | 0 | 6 (3.1) |
| Targeted therapy∗ | 1 | 1 | 2 | 0 | 4 (2) |
| Immune suppression with antiresorptive therapy | 0 | 0 | 1 | 0 | 1 (0.5) |
MS/MF RO−, multisystem/multifocal risk organ negative; RO+, risk organ positive; SS-pulm, single-system pulmonary; U, unifocal.
Vemurafenib, dabrafenib, cobimetinib, and an on-trial AKT inhibitor. Additional cases as second-line therapy.