Recommendations for treatment of MF stages IVA-IVB: first-line
| Treatment . | Comments* . |
|---|---|
| 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; autologous or allogeneic transplantation should be considered early in treatment paradigm for selected persons |
| TSEB and/or X-irradiation | Patients with advanced-stage disease may benefit from TSEB; “boosts” to site of thickened plaques/tumors; TSEB has limited availability; can take 6 to 10 weeks to complete; conventional radiation therapy can be valuable for local control of tumors or localized/bulky nodal disease |
| Bexarotene | See Table 7 for comments; few patients on clinical trials had stage IVB disease; thus, response rate and response durations are not well described |
| Denileukin diftitox | See Table 7 for comments; few patients on clinical trials had stage IVB disease; thus, response rate and response durations are not well described |
| IFN-α | See Table 7 for comments; less used in this stage of disease but may be helpful in patients unable to tolerate chemotherapy |
| Alemtuzumab | Major toxicity is immune suppression with infection; requires surveillance for cytomegalovirus and antimicrobial prophylaxis; short responses if used in multirelapsed disease so should consider early |
| Vorinostat | See Table 7 for comments; few patients on clinical trials had stage IVB disease; thus, response rate and response durations are not well described |
| Novel agents within clinical trials | Given poor prognosis and incurable nature of advanced-stage disease, it is very acceptable to consider novel agents within clinical trials before chemotherapy is considered (see Table 12) |
| Low-dose MTX | Generally well tolerated and convenient (oral weekly); dose-response effect is common and usually starts at 20 to 30 mg/week (up to 60-70 mg/week); some responses can be very durable; most common side effects are cytopenias and long-term risk of liver disease; very effective in patients with coexistent lymphomatoid papulosis; anecdotal experience that can be very useful in CD30+ MF or CD30+ transformed disease; can be used in conjunction with other therapies, such as steroids, ECP, and PUVA |
| Treatment . | Comments* . |
|---|---|
| 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; autologous or allogeneic transplantation should be considered early in treatment paradigm for selected persons |
| TSEB and/or X-irradiation | Patients with advanced-stage disease may benefit from TSEB; “boosts” to site of thickened plaques/tumors; TSEB has limited availability; can take 6 to 10 weeks to complete; conventional radiation therapy can be valuable for local control of tumors or localized/bulky nodal disease |
| Bexarotene | See Table 7 for comments; few patients on clinical trials had stage IVB disease; thus, response rate and response durations are not well described |
| Denileukin diftitox | See Table 7 for comments; few patients on clinical trials had stage IVB disease; thus, response rate and response durations are not well described |
| IFN-α | See Table 7 for comments; less used in this stage of disease but may be helpful in patients unable to tolerate chemotherapy |
| Alemtuzumab | Major toxicity is immune suppression with infection; requires surveillance for cytomegalovirus and antimicrobial prophylaxis; short responses if used in multirelapsed disease so should consider early |
| Vorinostat | See Table 7 for comments; few patients on clinical trials had stage IVB disease; thus, response rate and response durations are not well described |
| Novel agents within clinical trials | Given poor prognosis and incurable nature of advanced-stage disease, it is very acceptable to consider novel agents within clinical trials before chemotherapy is considered (see Table 12) |
| Low-dose MTX | Generally well tolerated and convenient (oral weekly); dose-response effect is common and usually starts at 20 to 30 mg/week (up to 60-70 mg/week); some responses can be very durable; most common side effects are cytopenias and long-term risk of liver disease; very effective in patients with coexistent lymphomatoid papulosis; anecdotal experience that can be very useful in CD30+ MF or CD30+ transformed disease; can be used in conjunction with other therapies, such as steroids, ECP, and PUVA |
For more details and detailed references, we refer the reader to the EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome.7