Table 4.
Treatments available by disease stage
| Stage or clinical presentation | First line | Second line |
|---|---|---|
| IA: T1, N0 | Expectant policy (T1a) Topical corticosteroids (T1a) Mechlorethamine gel Localized RT (localized MF including pagetoid reticulosis) UVB (T1a) Photodynamic therapy |
In addition with skin-directed treatments: • systemic therapies • retinoids (re-PUVA++) • IFNα • low-dose Mtx • TSEB (T2b) |
| IB: T2, N0 | Topical corticosteroids (T2a) Mechlorethamine gel UVB (T2a) PUVA |
|
| IIA: T1–2, N1–2 | PUVA Mechlorethamine | |
| IIB | Systemic therapies • retinoids • IFNα TSEB Monochemotherapy Low-dose Mtx±Localized (RT in combination) |
Polychemotherapy Allogeneic stem-cell transplantation • CD30-expressing: brentuximab vedotin |
| IIIA–B | Systemic therapies • Retinoids • IFNα TSEB Low-dose Mtx ECP (alone or combined) |
Monochemotherapy Allogeneic stem-cell transplantation |
| IVA–IVB (pertinent evidence insufficient to justify separation between first- and second-line treatment) | ECP stage IVA 4–A Chemotherapy Radiotherapy (TSEB and localized) Alemtuzumab (B2) Allogeneic stem-cell transplantation Mogamulizumab |
|
| Sézary syndrome | ECP retinoids or IFNα combined with ECP or PUVA Chlorambucil–prednisone Low-dose Mtx |
Chemotherapy Alemtuzumab Allogeneic stem-cell transplantation |
| Granulomatous slack skin | Localized RT | |
| Folliculotropic MF (FMF) | Same treatments according to stage, but poorer outcome: consider more aggressive treatment before unaggressive FMF: • topical steroids • phototherapy |
Advanced-stage FMF: • local radiotherapy • TSEB • PUVA combined with local radiotherapy |
| Children6 | Corticosteroids UVB PUVA |
|
Abbreviations: RT, radiotherapy; MF, mycosis fungoides; UV, ultraviolet; PUVA, psoralen with UVA; Mtx, methotrexate; TSEB, total-skin electron beam; ECP, extracorporeal photochemotherapy.