Treatment for Specific Types of Skin Lymphoma

The treatment you get for skin lymphoma will depend mainly on the type of lymphoma you have, its location, and its stage (how far it has spread). But other things can also affect your treatment options, such as your overall health.

The treatments mentioned here fall into 2 main groups:

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

Along with treatments aimed at the lymphoma itself, supportive care treatments for symptoms such as itching or skin infections are also an important part of care for many people with skin lymphomas. This type of treatment is often given by a dermatologist, a doctor who treats diseases of the skin.

Talk to your cancer care team if you have any questions about the treatment plan they recommend.

Mycosis fungoides (MF)

Many forms of treatment can be used for mycosis fungoides (MF).

Skin-directed treatments for MF

For early stages of MF, treatments are aimed at the skin. Sometimes more than one type of skin-directed treatment is used.

Options may include:

• Phototherapy with ultraviolet (UV) light, either UVB light or UVA combined with drugs called psoralens (known as PUVA)
• Topical chemotherapy with nitrogen mustard
• Topical corticosteroid ointments or injections
• Topical retinoids (vitamin A-like drugs), such as bexarotene
• Topical imiquimod (a form of immunotherapy)
• Local radiation treatments, if there are no more than a few lesions
• Total skin electron beam therapy (TSEBT), if MF covers most of the skin

To learn more about any of these treatments, see Skin-directed Treatments for Skin Lymphomas.

Systemic (whole-body) treatments for MF

MF might stay just in the skin for many years. But eventually it might spread, which could 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)
• Pembrolizumab (Keytruda)
• Low-dose methotrexate (a chemo drug)

To learn more about any of these treatments, see Whole-body (Systemic) Treatments for Skin Lymphomas.

Chemotherapy (usually with a single drug) or other medicines might also be an option. But these treatments are often reserved for lymphomas that are no longer responding to the treatments above.

If single chemo drugs are not effective, combinations of drugs might be recommended (similar to those used for other types of non-Hodgkin lymphoma).

Combining treatments for MF

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 (see above) 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).

An important goal of treatment for Sezary syndrome is the relief of symptoms related to the disease, such as itching.

Possible treatment options for Sezary syndrome

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 other drugs such as alemtuzumab or pembrolizumab (Keytruda) might 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.

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 isn’t causing symptoms, it can often be watched closely without needing to be treated right away. The skin lesions may even go away on their own, without any treatment.

Possible treatment options for C-ALCL (if needed)

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 skin lesions are in several places, the targeted drug brentuximab vedotin (Adcetris) or chemotherapy (often methotrexate, taken as a pill) is often the first treatment.
• Other chemotherapy, targeted therapy, or retinoid drugs might also be options, as well as radiation therapy (and possibly other skin-directed treatments).

If C-ALCL comes back or spreads

If the lymphoma comes back in the same place after treatment, the same treatment can often 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, brentuximab vedotin (Adcetris), chemotherapy, or a combination of the two might be an option. Sometimes radiation therapy might be given as well.

Lymphomatoid papulosis

This disease often comes and goes on its own. It usually has such a good outlook that treatment isn't needed right away, especially if the lesions aren't causing symptoms.

If treatment is needed, options depend on how extensive it is:

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

More intensive systemic therapies are rarely needed.

Subcutaneous panniculitis-like T-cell lymphoma

People with this rare type of skin lymphoma can live a long time and generally have an excellent outlook. The disease can often be controlled for long periods with corticosteroids or other medicines that suppress the immune system.

Chemotherapy, radiation, stem cell transplant, or newer treatments might also be options, if needed.

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 often 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 often be hard to control.
• Primary cutaneous acral CD8+ T-cell lymphoproliferative disorder tends to grow slowly, and it can usually be treated effectively with surgery or radiation therapy. It sometimes comes back, but it can often be treated again in the same way.
• Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder sometimes goes away on its own. If treatment is needed, it is usually surgery or radiation therapy, or a corticosteroid injected into the tumor. People with this lymphoma generally have a very 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 a good option.

Primary cutaneous marginal zone B-cell lymphoma OR primary cutaneous follicle center lymphoma

These types of skin lymphoma tend to have a good outlook. They can sometimes be watched without treatment until problems develop, but treatment is usually recommended.

Lymphomas 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, topical chemotherapy, bexarotene (Targretin), or imiquimod (Zyclara). Injected corticosteroids might also be used.

If the lymphoma doesn’t go away completely with one of these treatments, another one can be tried.

Lymphomas that have spread over larger parts of the skin

Treatment options include rituximab (Rituxan), injected corticosteroids, radiation therapy, or topical medicines such as corticosteroids, topical chemotherapy, bexarotene, or imiquimod.

If there are many lesions, systemic chemotherapy (sometimes with rituximab) can also be used, similar to treatment for other slow-growing B-cell lymphomas.

Lymphomas that have spread to lymph nodes or internal organs

In this case, the lymphoma is treated like follicular lymphoma or marginal zone lymphoma found in other parts of the body, such as with a combination of chemotherapy and rituximab.

To learn more, see Treating B-Cell Non-Hodgkin Lymphoma.

Primary cutaneous diffuse large B-cell lymphoma, leg type 

At first, these lymphomas might look like they involve only a small area of the skin, often on the legs. But the disease is often more widespread than it appears.

The treatment of choice is usually rituximab (Rituxan) along with systemic chemotherapy. Often the regimen called R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) is given, but other combinations can also be used.

If the lymphoma is in no more than a few areas, radiation therapy directed at the tumors is often used as well. For people who can’t get chemo for some reason, radiation therapy alone may be an option.

If the lymphoma has spread to lymph nodes or other organs

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.

To learn more, see Treating B-Cell Non-Hodgkin Lymphoma.

If the lymphoma keeps growing or comes back after treatment

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

When a cancer comes back after treatment, it is called recurrent or relapsed.

Lymphoma that comes back in the skin

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. Slow-growing skin lymphomas might just be watched closely unless they cause symptoms.

Lymphoma that comes back in another part of the body

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, spleen, or bone marrow. Different types of systemic treatments may be helpful in this situation.

• Chemotherapy might be used, especially if a person hasn’t had chemo before.
• Depending on the type of lymphoma and treatments a person had before, other options might include drugs such as vorinostat (Zolinza), romidepsin (Istodax), brentuximab vedotin (Adcetris), mogamulizumab (Poteligeo), or pembrolizumab (Keytruda).
• A stem cell transplant may be another option at some point.

Advanced skin lymphomas can be very hard to cure. Different systemic treatments may work for a while. But usually, the more treatments a person has, the less likely it is that the next treatment will help.

A good option for some people might be to consider entering a clinical trial testing a newer type of treatment. Many newer treatments are now being studied.