Treatment for specific types of skin lymphoma
The treatment of skin lymphoma is based mainly on the type of lymphoma, as well as its location and its stage – how far it has spread in the body. But other factors, such as your overall health, can also affect your treatment options. Talk to your doctor if you have any questions about the treatment plan he or she recommends.
The treatments mentioned in this section are discussed in more detail in earlier sections of this document.
Many forms of treatment can be used for mycosis fungoides (MF).
Skin-directed treatments: For early stages of MF, treatments are aimed at the skin. Options may include:
- Phototherapy with ultraviolet (UV) light (either UVB light or UVA combined with drugs called psoralens, known as PUVA)
- Topical chemotherapy with BCNU or nitrogen mustard
- Topical corticosteroid ointments or injections
- Topical retinoids (vitamin A-like drugs), such as bexarotene
- Topical imiquimod
- Local radiation treatments if there is only one or a few lesions
- Total skin electron beam therapy (TSEBT) if MF covers most of the skin
Sometimes more than one type of skin-directed treatment is used.
Systemic (whole-body) treatments: Mycosis fungoides might stay limited to the skin for many years. But eventually it might spread, and patients may need systemic treatments.
Several types of treatment can be used, such as:
- Retinoids (taken by mouth)
- Targeted drugs like vorinostat (Zolinza) or romidepsin (Istodax)
- Denileukin diftitox (Ontak)
- Low-dose methotrexate (a chemo drug)
Chemotherapy (usually with a single drug) or alemtuzumab (Campath) might be other options, but they are often reserved for lymphomas that are no longer responding to other treatments. If single chemo drugs are not effective, combinations of drugs (similar to those used for other types of non-Hodgkin lymphoma) might be recommended.
More than one type of treatment might be used at the same time. This could include combinations of skin-directed and systemic treatments (such as TSEBT plus photopheresis) or combined systemic treatments (such as an oral retinoid plus interferon).
Many patients can be helped by these treatments, sometimes for many years, but they rarely cure the lymphoma. If other treatments are no longer working, a stem cell transplant may be an option. Newer treatments are also being studied. If current treatments are no longer helpful, patients may want to consider entering a clinical trial.
The systemic treatments used for advanced MF are also used to treat Sezary syndrome. This disease has usually spread beyond the skin at the time it is diagnosed, so treatments directed only at the skin are less useful than in MF (although some might still be part of treatment).
Photopheresis may be helpful in treating the disease, as may retinoids, such as bexarotene. The targeted treatments vorinostat and romidepsin might also be used, as might interferon or denileukin diftitox. Chemotherapy or alemtuzumab can also be useful, but these are usually reserved for lymphomas that are no longer responding to other treatments. A stem cell transplant might be another option if other treatments are no longer working.
As with advanced MF, these treatments are often helpful for a time, but they rarely result in a cure. Newer treatments are now being studied, and patients may want to consider entering a clinical trial of one of these.
Primary cutaneous anaplastic large cell lymphoma (ALCL)
This lymphoma usually stays confined to the skin. It seldom spreads inside the body and rarely causes death. If it’s not causing symptoms, it can often be monitored closely without needing to be treated right away. The skin lesions may even go away on their own, without any treatment.
If treatment is needed, surgery and/or radiation therapy are the most common options for single skin lesions (or small groups of lesions). Topical chemotherapy, retinoids, or other medicines might be options if there are skin lesions in several places.
If the lymphoma comes back after treatment and spreads to lymph nodes or (rarely) internal organs, then systemic chemotherapy is often used. Newer targeted drugs such as brentuximab vedotin (Adcetris) and crizotinib (Xalkori) have been shown help some patients with non-skin forms of ALCL. These might also be options for advanced cutaneous ALCL, although more research is needed.
This disease often comes and goes on its own and usually has such a good outlook that treatment is not needed right away. If treatment is needed, phototherapy and topical corticosteroids are the most common treatments if there are only a few skin lesions. If the lesions are more extensive, topical chemotherapy or systemic treatments such as oral retinoids or low-dose methotrexate are other options. Rarely is there any need for systemic chemotherapy.
Subcutaneous panniculitis-like T-cell lymphoma
Patients with this type of lymphoma can live a long time and generally have an excellent outlook. Although chemotherapy and radiation have been used successfully in the past, the disease can often be controlled for long periods with just corticosteroids.
Primary cutaneous peripheral T-cell lymphoma, unspecified
Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphomas are usually fast growing and are treated with systemic chemotherapy.
Primary cutaneous gamma/delta T-cell lymphoma tends to grow and spread very quickly. It is treated with systemic chemotherapy or radiation therapy, but generally does not respond well to treatment.
Primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma may be removed with surgery or treated with radiation if there is only a single tumor. If there are many tumors, systemic chemotherapy or corticosteroids are often effective. People with this lymphoma generally have a good outlook, especially if they have only one tumor.
Primary cutaneous peripheral T-cell lymphoma, unspecified, is treated with systemic chemotherapy. Although these lymphomas may respond to chemotherapy at first, they often come back later, at which point they can be very hard to treat.
These lymphomas are often hard to treat effectively, so patients may want to consider clinical trials studying newer forms of treatment.
Primary cutaneous marginal-zone B-cell lymphoma or
Primary cutaneous follicle-center lymphoma
These types of lymphoma can sometimes be watched without treatment until problems develop. For lymphomas that are in one spot or only a few spots close together, initial treatment is usually radiation therapy or surgery. If the lymphoma does not go away completely or keeps growing, further treatment may include surgery; radiation therapy; topical medicines such as corticosteroids, chemotherapy, bexarotene (Targretin), or imiquimod (Zyclara); or injected corticosteroids.
For lymphomas that have spread over larger parts of the skin, treatment options include rituximab (Rituxan), topical medicines (such as corticosteroids, chemotherapy, bexarotene, or imiquimod), radiation therapy, or injected corticosteroids. Systemic chemotherapy (sometimes with rituximab), like that used for other slow-growing B-cell lymphomas, can also be used if there are many lesions.
If the lymphoma has spread to lymph nodes or internal organs, it is treated like follicular lymphomas found in other parts of the body, typically with a combination of chemotherapy and rituximab (see Non-Hodgkin Lymphoma for more details).
Primary cutaneous diffuse large B-cell lymphoma, leg type
These lymphomas might look like they involve only a small area of the skin at first, but the disease is often more widespread than it first appears. The treatment of choice is rituximab along with systemic chemotherapy. Often the regimen called R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) is given, but other chemo combinations can also be used. If the lymphoma is in only one or a few areas, radiation therapy directed at the tumors is often used as well. For people who can’t tolerate chemotherapy, radiation therapy alone may be given.
If the lymphoma has spread to the lymph nodes or other organs, treatment is the same as that used for diffuse large B-cell lymphomas (DLBCLs) found in other parts of the body, which is usually R-CHOP, with or without radiation therapy (see Non-Hodgkin Lymphoma for more details).
Primary cutaneous diffuse large B-cell lymphoma, other (non-leg)
Patients with this type of lymphoma (which involves sites other than the leg) need systemic chemotherapy, similar to that used for primary cutaneous diffuse large B-cell lymphoma, leg type. This is most often the R-CHOP regimen (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone).
Last Medical Review: 08/04/2014
Last Revised: 02/24/2016