Recommendations for treatment of stage III or SS (stages III or IVa)
| Treatment . | Comments* . |
|---|---|
| First-line | |
| ECP | Well tolerated with limited toxicities; circulating T-cell clone should be detectable in blood by morphology, flow cytometry, or molecular studies; should not be considered in patients with SS who have extensive nodal (IVa) or visceral (IVb) disease; side effects to methoxsalen is rare; requires good venous access with the associated risk of infection; often combined with oral steroids (short-term), IFN-α, bexarotene, or low-dose MTX; improvement with ECP alone can take some weeks and maximum improvement may not be seen for many months; durable responses are not uncommon |
| IFN-α | Major difficulty is tolerance and compliance; some responses can be very durable; somewhat inconvenient (daily subcutaneous injection); most common side effect is fatigue, particularly in older patients; requires moderately high doses aiming for 3 to 5+ MU/day; monitor FBC and thyroid function; IFN-α can also be combined with PUVA, retinoids, bexarotene, and ECP |
| PUVA + IFN-α | For stage III disease; would not generally recommend PUVA alone; requires regular 2 or 3 times/week treatment and limited number of sites in nonmetropolitan areas |
| MTX | See Table 7 for comments |
| Second-line | |
| Bexarotene | See Table 7 for comments; can consider adding to ECP or IFN-α |
| Vorinostat | See Table 7 for comments; no data available of adding to ECP or IFN-α |
| Denileukin diftitox | See Table 7 for comments |
| Alemtuzumab | See Table 10 for comments |
| Novel agents within clinical trials | In patients with SS, chemotherapy is recommended after bexarotene and/or and HDACi and/or DD; it is very acceptable to consider novel agents within clinical trials before chemotherapy is considered (see Table 12) |
| Chemotherapy | Choice of chemotherapy regimens is extensive (see Table 11), and choice depends on patient tolerance, risk of infection versus the relatively short duration of remission observed with most chemotherapy regimens; transplantation may be considered in highly selected individuals |
| Treatment . | Comments* . |
|---|---|
| First-line | |
| ECP | Well tolerated with limited toxicities; circulating T-cell clone should be detectable in blood by morphology, flow cytometry, or molecular studies; should not be considered in patients with SS who have extensive nodal (IVa) or visceral (IVb) disease; side effects to methoxsalen is rare; requires good venous access with the associated risk of infection; often combined with oral steroids (short-term), IFN-α, bexarotene, or low-dose MTX; improvement with ECP alone can take some weeks and maximum improvement may not be seen for many months; durable responses are not uncommon |
| IFN-α | Major difficulty is tolerance and compliance; some responses can be very durable; somewhat inconvenient (daily subcutaneous injection); most common side effect is fatigue, particularly in older patients; requires moderately high doses aiming for 3 to 5+ MU/day; monitor FBC and thyroid function; IFN-α can also be combined with PUVA, retinoids, bexarotene, and ECP |
| PUVA + IFN-α | For stage III disease; would not generally recommend PUVA alone; requires regular 2 or 3 times/week treatment and limited number of sites in nonmetropolitan areas |
| MTX | See Table 7 for comments |
| Second-line | |
| Bexarotene | See Table 7 for comments; can consider adding to ECP or IFN-α |
| Vorinostat | See Table 7 for comments; no data available of adding to ECP or IFN-α |
| Denileukin diftitox | See Table 7 for comments |
| Alemtuzumab | See Table 10 for comments |
| Novel agents within clinical trials | In patients with SS, chemotherapy is recommended after bexarotene and/or and HDACi and/or DD; it is very acceptable to consider novel agents within clinical trials before chemotherapy is considered (see Table 12) |
| Chemotherapy | Choice of chemotherapy regimens is extensive (see Table 11), and choice depends on patient tolerance, risk of infection versus the relatively short duration of remission observed with most chemotherapy regimens; transplantation may be considered in highly selected individuals |
FBC indicates fludarabine, busulphan, and alemtuzumab.
For more details and detailed references, we refer the reader to the EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome.7