Recommendations for first-line 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 either 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 sc injection). Most common side effect is fatigue particularly in older patients. Requires moderately high doses aiming for 3-5+ MU/d. 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 2-3×/wk treatment and limited number of sites in nonmetropolitan areas. |
| Bexarotene | See Table 1 for comments. Can consider adding to ECP or IFN-α. |
| MTX | See Table 1 for comments. |
| Second-line | |
| Alemtuzumab | See Table 1 for comments. Can be considered first line in suitable patients. |
| Vorinostat/romidepsin | See Table 1 for comments. No data available of adding to ECP or IFN-α. |
| Pralatrexate | See Table 1 for comments. |
| Allogeneic transplant | For suitable patients. |
| Novel agents and clinical trials | In patients with SS, chemotherapy is recommended after bexarotene and/or and HDACi and/or DD. It is very acceptable to consider a clinical trials/novel agents before chemotherapy is considered. |
| Chemotherapy | Choice of chemotherapy regimens is extensive and choice depends on patient tolerance, risk of infection vs 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 either 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 sc injection). Most common side effect is fatigue particularly in older patients. Requires moderately high doses aiming for 3-5+ MU/d. 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 2-3×/wk treatment and limited number of sites in nonmetropolitan areas. |
| Bexarotene | See Table 1 for comments. Can consider adding to ECP or IFN-α. |
| MTX | See Table 1 for comments. |
| Second-line | |
| Alemtuzumab | See Table 1 for comments. Can be considered first line in suitable patients. |
| Vorinostat/romidepsin | See Table 1 for comments. No data available of adding to ECP or IFN-α. |
| Pralatrexate | See Table 1 for comments. |
| Allogeneic transplant | For suitable patients. |
| Novel agents and clinical trials | In patients with SS, chemotherapy is recommended after bexarotene and/or and HDACi and/or DD. It is very acceptable to consider a clinical trials/novel agents before chemotherapy is considered. |
| Chemotherapy | Choice of chemotherapy regimens is extensive and choice depends on patient tolerance, risk of infection vs the relatively short duration of remission observed with most chemotherapy regimens. Transplantation may be considered in highly selected individuals. |