Recommendations for treatment of MF stages IA, IB, and IIA
| Treatment . | Comments* . |
|---|---|
| First-line | |
| “Expectant policy” | Usually suitable for those with stage IA disease in conjunction with symptomatic treatment if required; patients with single lesion may be considered for “curative therapy” with radiation therapy |
| PUVA | For patch/plaque disease; requires regular 2 or 3 times/week treatment; there may be limited availability of PUVA in nonmetropolitan areas; can be combined with retinoids/rexinoids |
| UVB | For patch stage disease as skin penetration not as deep as PUVA; requires regular 2 or 3 times/week treatment and generally more readily available than PUVA |
| Topical corticosteroids | Simple therapy; toxicities if extensive skin application for long periods |
| Topical bexarotene | For limited sites of disease; simple therapy; local reactions may occur |
| Topical NM | For limited sites of disease or generalized involvement; local reactions occasionally problematic; ointment causes fewer reactions; availability of NM worldwide has been a problem recently |
| Topical carmustine | Rarely used now; for limited sites of disease; local reactions may occur; causes telangiectasias |
| Localized radiotherapy | Especially for patients with limited number of lesions and/or thickened plaques; durable remissions achieved |
| TSEB | Patients with stage IB disease with relatively slow progression; limited availability; can take 6 to 10 weeks to complete |
| Second-line+ | |
| Oral bexarotene | Generally well tolerated and convenient (oral capsule); some responses can be very durable; most common side effects are hypertriglyceridemia and hypothyroidism that usually require treatment; other relatively common side effects are rash and headache; can be used in conjunction with other therapies |
| IFN-α monotherapy | 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 |
| 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; can be used in conjunction with other therapies, such as steroids, ECP, PUVA, IFN-α |
| Vorinostat | Only approved HDACi currently; generally well tolerated and convenient (oral daily); there appears to be a dose-response effect in some patients; most common SEs are fatigue, lethargy, mild/moderate thrombocytopenia and elevated creatinine and taste changes; can improve itch even when skin lesions remain; some responses can be very durable; virtually no data on use in combination with other therapies, such as PUVA, IFN-α, MTX, chemotherapy |
| Denileukin diftitox | Generally considered after trial of bexarotene and/or HDACi; inconvenient administration requiring daily dosing times 5 days every 3 weeks (6-8 courses); patient's tumor must express CD25 (although responses are observed in patients with CD25− lesions); there can be substantial supportive care requirements for some patients during therapy who develop capillary leak syndrome; some responses can be very durable even in heavily pretreated patients |
| Novel agents within clinical trials | In patients with stage IA-IIA disease, chemotherapy is not recommended and novel agents within clinical trials are generally recommended before chemotherapy is considered (see Table 12) |
| Treatment . | Comments* . |
|---|---|
| First-line | |
| “Expectant policy” | Usually suitable for those with stage IA disease in conjunction with symptomatic treatment if required; patients with single lesion may be considered for “curative therapy” with radiation therapy |
| PUVA | For patch/plaque disease; requires regular 2 or 3 times/week treatment; there may be limited availability of PUVA in nonmetropolitan areas; can be combined with retinoids/rexinoids |
| UVB | For patch stage disease as skin penetration not as deep as PUVA; requires regular 2 or 3 times/week treatment and generally more readily available than PUVA |
| Topical corticosteroids | Simple therapy; toxicities if extensive skin application for long periods |
| Topical bexarotene | For limited sites of disease; simple therapy; local reactions may occur |
| Topical NM | For limited sites of disease or generalized involvement; local reactions occasionally problematic; ointment causes fewer reactions; availability of NM worldwide has been a problem recently |
| Topical carmustine | Rarely used now; for limited sites of disease; local reactions may occur; causes telangiectasias |
| Localized radiotherapy | Especially for patients with limited number of lesions and/or thickened plaques; durable remissions achieved |
| TSEB | Patients with stage IB disease with relatively slow progression; limited availability; can take 6 to 10 weeks to complete |
| Second-line+ | |
| Oral bexarotene | Generally well tolerated and convenient (oral capsule); some responses can be very durable; most common side effects are hypertriglyceridemia and hypothyroidism that usually require treatment; other relatively common side effects are rash and headache; can be used in conjunction with other therapies |
| IFN-α monotherapy | 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 |
| 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; can be used in conjunction with other therapies, such as steroids, ECP, PUVA, IFN-α |
| Vorinostat | Only approved HDACi currently; generally well tolerated and convenient (oral daily); there appears to be a dose-response effect in some patients; most common SEs are fatigue, lethargy, mild/moderate thrombocytopenia and elevated creatinine and taste changes; can improve itch even when skin lesions remain; some responses can be very durable; virtually no data on use in combination with other therapies, such as PUVA, IFN-α, MTX, chemotherapy |
| Denileukin diftitox | Generally considered after trial of bexarotene and/or HDACi; inconvenient administration requiring daily dosing times 5 days every 3 weeks (6-8 courses); patient's tumor must express CD25 (although responses are observed in patients with CD25− lesions); there can be substantial supportive care requirements for some patients during therapy who develop capillary leak syndrome; some responses can be very durable even in heavily pretreated patients |
| Novel agents within clinical trials | In patients with stage IA-IIA disease, chemotherapy is not recommended and novel agents within clinical trials are generally recommended before chemotherapy is considered (see Table 12) |
For more details and detailed references, we refer the reader to the EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome.7