Table 8.
Recommendations of the Cutaneous Lymphomas Group of the Dermatology and Hematology Divisions of HC-FMUSP for the treatment of mycosis fungoides and Sézary syndrome.
| CS | First-line | Second line | Third line |
|---|---|---|---|
| Ia | Topical corticosteroids | Localized topic PUVA | Localized radiotherapy (if regionalized disease) |
| Phototherapy with NB-UVB | PUVA | ||
| Topical mechlorethaminea | |||
| Topical carmustine | |||
| Bexarotene gela | |||
| Imiquimod / Resiquimod | |||
| Ib, IIa | Phototherapy with NB-UVB | Systemic retinoidsb ± phototherapy with NB-UVB or PUVA | Total skin electron beam radiotherapy |
| PUVA phototherapy | Alpha interferon ± phototherapy with NB-UVB or PUVA | Low-dose methotrexate | |
| IIb | Systemic retinoids ± phototherapy with NB-UVB or PUVA ± localized RT | Total skin electron beam radiotherapy | Monochemotherapy (gemcitabine, pegylated liposomal doxorubicin) |
| Low-dose methotrexate | |||
| Brentuximab vedotin (if CD30-positive) | HDACia | ||
| Alpha-interferon ± phototherapy with NB-UVB or PUVA ± localized RT | Mogamulizumab | ||
| Polychemotherapy (CHOP type) | |||
| Allogeneic hematopoietic stem cell transplantation | |||
| IIIa, IIIb | Systemic retinoidsb ± PUVA | Chlorambucil + prednisone | HDACia |
| Alpha interferon ± PUVA | Low-dose methotrexate | Polychemotherapy (CHOP type) | |
| ECP ± systemic retinoidsb ± alpha-interferon | Monochemotherapy (gemcitabine, pegylated liposomal doxorubicin) | Allogeneic hematopoietic stem cell transplantation | |
| IVa1, SS | ECP ± alpha-interferon ± systemic retinoidsb | Chlorambucil + prednisone | Monochemotherapy |
| Low-dose methotrexate | Polychemotherapy (CHOP type) | ||
| Alpha interferon ± PUVA | Alemtuzumab | Allogeneic hematopoietic stem cell transplantation | |
| Mogamulizumaba | |||
| IVa2 | Monochemotherapy (gemcitabine, pegylated liposomal doxorubicin) | Brentuximab vedotin (if CD30-positive) | HDACia |
| Polychemotherapy (CHOP type) | |||
| Radiotherapy if localized nodal disease | Allogeneic hematopoietic stem cell transplantation | ||
| IVb | Monochemotherapy (gemcitabine, pegylated liposomal doxorubicin) | Brentuximab vedotin (if CD30-positive) | Polychemotherapy (CHOP type) |
| Allogeneic hematopoietic stem cell transplantation |
HC-FMUSP, Hospital de Clínicas, Faculdade de Medicina, Universidade de São Paulo; CS, clinical-stage; PUVA, psoralen + ultraviolet A; NB-UVB, narrow-band UVB; RT, radiotherapy; HDACi, histone deacetylase inhibitors; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisolone; SS, Sézary syndrome; ECP, extracorporeal photopheresis.
a
Agent not available in Brazil.
b
Bexarotene is not available in Brazil, being replaced by acitretin and, less commonly, by isotretinoin.