Summary of treatment options for MF/SS
| Therapy . | MF . | SS/E-MF . | Comment including potential toxicities . | |
|---|---|---|---|---|
| Early-stage disease . | Advanced-stage disease . | |||
| “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 RT. | ||
| Topical corticosteroids | ++++ | ++ | +++ | Simple therapy. Toxicities if extensive skin application for long periods. |
| PUVA | +++ | + | +++ | For patch/plaque disease. Requires regular 2-3×/wk treatment. There may be limited availability of PUVA in nonmetropolitan areas. Can be combined with retinoids/rexinoids. Risk of skin cancers with cumulative dosing. |
| UVB (TLO1) | ++++ | + | ++ | For patch stage disease as skin penetration not as deep as PUVA. Requires regular 2-3×/wk treatment and generally more readily available than PUVA. Risk of skin cancers with cumulative dosing. |
| Topical chemotherapy | ++ | If limited number of lesions. For limited sites of disease. Local reactions occasionally problematic. | ||
| Imiquimod | + | If small lesions and limited number of lesions. Can cause inflammatory reactions. | ||
| Photodynamic therapy | + | If limited number of lesions. Limited availability. | ||
| Retinoids | + | + | + | Usually second line. Less used since bexarotene became available. |
| Bexarotene | ++ | +++ | ++++ | Usually second line. 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 such as PUVA or IFN-α and ECP for E-MF/SS. Topical form for limited sites of disease but local reactions may occur. |
| Interferon α | ++ | +++ | ++++ | Second line; major difficulty is tolerance and compliance. Some responses can be very durable. Somewhat inconvenient (daily sc injection). Most common side effect is fatigue, anorexia, and mood changes particularly in older patients. Monitoring for cytopenias and thyroid disturbance is recommended. Requires moderately high doses aiming for 3-5+ MU/d or 3×/wk. Monitor FBC and thyroid function. IFN-α can also be combined with PUVA, retinoids, bexarotene. |
| HDACi: vorinostat, romidepsin | ++ | +++ | ++++ | Beyond second line. 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. Limited data on use in combination with other therapies. |
| Oral methotrexate | + | +++ | +++ | Low dose weekly. Generally well tolerated and convenient (oral weekly). Dose-response effect is common and usually start at 20-30 mg/wk (up to 60-70 mg/wk). 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. |
| Localized radiotherapy | +++ | +++ | If localized or large/plaques and tumor nodules. | |
| TSEB | + | ++ | + | For widespread disease. Higher doses associated with acute skin toxicities. Can be repeated but high cumulative doses can result in skin toxicity. |
| Systemic chemotherapy | ++ | ++ | Beyond second line. | |
| ECP | ++++ | Variable availability. Venous access can be problematic. | ||
| Pralatrexate | +++ | + | Beyond second line. Generally well tolerated. Mucositis is most common toxicity. Supportive measures with B12 supplementation required. Dose modification generally recommended for MF (lower dose than PTCL). | |
| Allogeneic transplantation | + | ++ | Very selected cases. | |
| Denileukin diftitox | ++ | ++ | Beyond second line (availability limited currently). Major toxicity is capillary leak syndrome which can be severe. | |
| Alemtuzumab | + | ++ | Second line or beyond. More effective in SS/E-MF. Higher doses are associated with CMV reactivation. | |
| Immunomodulatory agents (lenalidomide) | + | Beyond second line. Has not achieved regulatory approval. Fatigue, venous thromboembolism, and cytopenias are the most common toxicities. | ||
| Proteasome inhibitors | + | Under investigation. Has not achieved regulatory approval. Cytopenias and neuropathy (bortezomib) can occur. | ||
| Novel agents and clinical trials | Chemotherapy generally only achieves short remissions and clinical trials/novel agents can be recommended before chemotherapy is considered. | |||
| Therapy . | MF . | SS/E-MF . | Comment including potential toxicities . | |
|---|---|---|---|---|
| Early-stage disease . | Advanced-stage disease . | |||
| “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 RT. | ||
| Topical corticosteroids | ++++ | ++ | +++ | Simple therapy. Toxicities if extensive skin application for long periods. |
| PUVA | +++ | + | +++ | For patch/plaque disease. Requires regular 2-3×/wk treatment. There may be limited availability of PUVA in nonmetropolitan areas. Can be combined with retinoids/rexinoids. Risk of skin cancers with cumulative dosing. |
| UVB (TLO1) | ++++ | + | ++ | For patch stage disease as skin penetration not as deep as PUVA. Requires regular 2-3×/wk treatment and generally more readily available than PUVA. Risk of skin cancers with cumulative dosing. |
| Topical chemotherapy | ++ | If limited number of lesions. For limited sites of disease. Local reactions occasionally problematic. | ||
| Imiquimod | + | If small lesions and limited number of lesions. Can cause inflammatory reactions. | ||
| Photodynamic therapy | + | If limited number of lesions. Limited availability. | ||
| Retinoids | + | + | + | Usually second line. Less used since bexarotene became available. |
| Bexarotene | ++ | +++ | ++++ | Usually second line. 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 such as PUVA or IFN-α and ECP for E-MF/SS. Topical form for limited sites of disease but local reactions may occur. |
| Interferon α | ++ | +++ | ++++ | Second line; major difficulty is tolerance and compliance. Some responses can be very durable. Somewhat inconvenient (daily sc injection). Most common side effect is fatigue, anorexia, and mood changes particularly in older patients. Monitoring for cytopenias and thyroid disturbance is recommended. Requires moderately high doses aiming for 3-5+ MU/d or 3×/wk. Monitor FBC and thyroid function. IFN-α can also be combined with PUVA, retinoids, bexarotene. |
| HDACi: vorinostat, romidepsin | ++ | +++ | ++++ | Beyond second line. 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. Limited data on use in combination with other therapies. |
| Oral methotrexate | + | +++ | +++ | Low dose weekly. Generally well tolerated and convenient (oral weekly). Dose-response effect is common and usually start at 20-30 mg/wk (up to 60-70 mg/wk). 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. |
| Localized radiotherapy | +++ | +++ | If localized or large/plaques and tumor nodules. | |
| TSEB | + | ++ | + | For widespread disease. Higher doses associated with acute skin toxicities. Can be repeated but high cumulative doses can result in skin toxicity. |
| Systemic chemotherapy | ++ | ++ | Beyond second line. | |
| ECP | ++++ | Variable availability. Venous access can be problematic. | ||
| Pralatrexate | +++ | + | Beyond second line. Generally well tolerated. Mucositis is most common toxicity. Supportive measures with B12 supplementation required. Dose modification generally recommended for MF (lower dose than PTCL). | |
| Allogeneic transplantation | + | ++ | Very selected cases. | |
| Denileukin diftitox | ++ | ++ | Beyond second line (availability limited currently). Major toxicity is capillary leak syndrome which can be severe. | |
| Alemtuzumab | + | ++ | Second line or beyond. More effective in SS/E-MF. Higher doses are associated with CMV reactivation. | |
| Immunomodulatory agents (lenalidomide) | + | Beyond second line. Has not achieved regulatory approval. Fatigue, venous thromboembolism, and cytopenias are the most common toxicities. | ||
| Proteasome inhibitors | + | Under investigation. Has not achieved regulatory approval. Cytopenias and neuropathy (bortezomib) can occur. | ||
| Novel agents and clinical trials | Chemotherapy generally only achieves short remissions and clinical trials/novel agents can be recommended before chemotherapy is considered. | |||
Crosses indicate approximate frequency of use.
CMV, cytomegalovirus; FBC, full blood count; PTCL, peripheral T-cell lymphoma; RT, radiotherapy; sc, subcutaneous; SE, side effects.