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. 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 fall into 2 main groups:

• Skin-directed treatments
• Whole-body (systemic) treatments

T-cell lymphomas

Mycosis fungoides (MF)

Many forms of treatment can be used for 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 just in the skin for many years. But eventually it might spread, which might require systemic treatment. Several types of treatment can be used, such as:

• Retinoids (taken by mouth)
• Targeted drugs like vorinostat (Zolinza) or romidepsin (Istodax)
• Photopheresis
• Interferons
• Brentuximab vedotin (Adcetris)
• Mogamulizumab (Poteligeo)
• Low-dose methotrexate (a chemo drug)

Chemotherapy (usually with a single drug) or other medicines might be other options, but they are often reserved for lymphomas that are no longer responding to the treatments above. 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 people 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, so it might be worth considering entering a clinical trial.

Sezary syndrome

The systemic treatments used for advanced MF are also used to treat Sezary syndrome. This disease usually has 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 drugs vorinostat (Zolinza) and romidepsin (Istodax) might also be used, as might interferon, brentuximab vedotin (Adcetris), or mogamulizumab (Poteligeo). 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, so it might be worth considering entering a clinical trial of one of these.

Primary cutaneous anaplastic large cell lymphoma (C-ALCL)

This lymphoma usually stays confined to the skin. It can come back after treatment, but it seldom spreads inside the body and is rarely fatal. 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, options depend on how extensive the lymphoma is:

• For single skin lesions (or small groups of lesions), surgery and/or radiation therapy are the most common options. 
• If there are skin lesions in several places, chemotherapy (often methotrexate, taken as a pill) or the targeted drug brentuximab vedotin (Adcetris) are typical first options. Other chemotherapy, targeted therapy, or retinoid drugs might also be options, as well as radiation therapy.

If the lymphoma comes back in the same place after treatment, often the same treatment can be used again. If one treatment is no longer helpful, another can be tried.

If the lymphoma spreads to the lymph nodes or (rarely) internal organs, then systemic chemotherapy or brentuximab vedotin (Adcetris) is often used. Radiation therapy might also be an option.

Lymphomatoid papulosis

This disease often comes and goes on its own and usually has such a good outlook that treatment isn't needed right away, especially if the lesions aren't causing any symptoms. If treatment is needed, options depend on how extensive the lymphoma is:

• If there are only a few skin lesions, phototherapy and topical corticosteroids are the most common treatments.
• If the lesions are more extensive, skin-directed treatments (such as phototherapy or topical chemotherapy or retinoids) or systemic treatments (such as oral retinoids or low-dose methotrexate) are other options.

More intensive systemic therapies are rarely needed.

Subcutaneous panniculitis-like T-cell lymphoma

Patients with this rare 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, rare subtypes

Primary cutaneous gamma/delta T-cell lymphoma tends to grow and spread very quickly. It is treated with systemic chemotherapy using a combination of drugs, but even with treatment it can be hard to control.

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma usually grows quickly and is treated with systemic chemotherapy using a combination of drugs. Even with treatment, it can be hard to control.

Primary cutaneous acral CD8+ T-cell lymphoma tends to grow slowly, and can usually be treated effectively with surgery or radiation therapy. It can sometimes come back, but it can often be treated again in a similar manner.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder sometimes goes away on its own. If treatment is needed, it can usually be done with surgery or radiation therapy, or by injecting a corticosteroid into the tumor. People with this lymphoma generally have a good outlook, especially if they have only one tumor.

Some of these lymphomas can be hard to treat effectively, so clinical trials studying newer forms of treatment might be good options.

B-cell lymphomas

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, but usually treatment is recommended.

For lymphomas that are in one spot or only a few spots close together, initial treatment is usually radiation therapy or surgery. Other options might include topical medicines such as corticosteroids, chemotherapy, bexarotene (Targretin), or imiquimod (Zyclara); or injected corticosteroids. If the lymphoma does not go away completely, one of the other treatments can be tried.

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), injected corticosteroids, or radiation therapy. 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 (Rituxan) 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).

If the lymphoma keeps growing or comes back after treatment

Some skin lymphomas respond well to treatment, but others might not. If this happens, other types of treatment can often be tried. But as more treatments are tried, they may be less likely to work or more likely to cause side effects.

When a cancer comes back after treatment it is called recurrent or relapsed. In general, if a skin lymphoma comes back it tends to be in the skin. If this is the case, skin-directed therapies that haven’t been used yet may be effective.

Some skin lymphomas eventually spread inside the body as well. Often, the lymph nodes are the first site of relapse. After that, the lymphoma might spread to organs such as the liver or spleen, and bone marrow. Different types of systemic treatments may be helpful in this situation. Chemotherapy is often used, especially if a person hasn’t had chemo before. Depending on the type of lymphoma and which treatments a person has had before, other drugs, such as vorinostat (Zolinza), romidepsin (Istodax), brentuximab vedotin (Adcetris), or mogamulizumab (Poteligeo), might also be options. A stem cell transplant may be another option at some point.

Advanced skin lymphomas are very hard to cure. Different systemic treatments may be effective for some time. But in general, the more treatments a person has had, the less likely it is that the next treatment will be helpful.

A good option for some people might be to consider clinical trials of treatments that work in new ways. Many newer treatments are now being studied. For more info, see What’s New in Skin Lymphoma Research? 

If newer or more aggressive treatments are no longer working, at some point a person might want to think about treatments aimed more at relieving the symptoms of the lymphoma. This approach is called palliative care.