What is cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma (CTCL) is a rare type of cancer that affects the skin. It starts in blood cells called T-lymphocytes. These are white blood cells that are part of your immune system. They normally fight infection. They can be found in lymph tissue all over the body, such as in the spleen, tonsils, intestines, and skin. CTCL develops when abnormal T cells grow in the skin.

CTCL causes itchy, scaly rashes, round patches, or bumps that can thicken to form lesions or tumors. This cancer is also known as lymphoma of the skin. It’s a type of non-Hodgkin lymphoma.

CTCL is often a slow-growing cancer. It develops over many years. 

The two most common types of this cancer are mycosis fungoides (MF) and Sézary syndrome (SS).

What causes cutaneous T-cell lymphoma?

Experts aren’t exactly sure what causes cutaneous T-cell lymphoma. But it occurs when T-lymphocytes in the body change and grow out of control. These abnormal cells may grow to form a lump or mass called a tumor. The tumor can grow into (invade) nearby areas. It can even spread to other parts of the body (metastasis).

Who is at risk for cutaneous T-cell lymphoma?

A risk factor is anything that may increase your chance of having a disease. The exact cause of someone’s cancer may not be known. But risk factors can make it more likely for a person to have cancer. Some risk factors may not be in your control. But others may be things you can change. 

Anyone can get CTCL. Experts aren’t exactly sure what causes it. But certain factors that might raise your risk are:

• Being older

• Being male

• Having a weakened immune system, such as from AIDS or an organ transplant

• Having certain infections, such as the Epstein-Barr virus

Talk with your healthcare provider about your risk factors for CTCL and what you can do about them.

What are the symptoms of cutaneous T-cell lymphoma?

The symptoms of CTCL depend on how much of the skin is affected by the cancer. These symptoms can look a lot like other skin conditions. Make sure to see your healthcare provider for a diagnosis. The most common symptoms of CTCL are:

• Skin changes. Patches, thick lesions (plaques), or bumps form on the skin. They’re dry, itchy, red, and scaly. The lesions can also be purple or brown in color or lighter than the skin around it. As the cancer spreads, these patches will cover more of the skin surface. They may also grow in size. They may thicken so that tumors form on the skin. MF tends to grow slowly. SS may cause skin redness over most of the body (erythroderma).

• Enlarged lymph nodes. In early stages of the cancer, lymph nodes are normal in size. But they become larger as the cancer spreads. In later stages, cancer cells from the skin may have spread into the lymph nodes, blood, and other organs.

Many of these changes may be caused by other health problems. But it’s important to see your healthcare provider if you have these symptoms. Only a healthcare provider can tell if you have cancer.

How is cutaneous T-cell lymphoma diagnosed?

The most common way to find CTCL is when a person sees a healthcare provider because of an itchy patch or rash on their skin that doesn’t get better or keeps getting bigger. The provider will ask about your health history, symptoms, risk factors, and family history of disease. They will do a physical exam and check for enlarged lymph nodes and look at all of your skin. 

You likely will be sent to a dermatologist. This is a healthcare provider with special training to treat skin problems. The healthcare provider may use a special light, magnifying lens, or camera to get a very close look at the changed skin. 

You will very likely need one or more skin biopsies. This is when the healthcare provider takes small pieces of tissue from the affected skin. These samples can be removed with a sharp blade or scalpel and looked at under a microscope by a pathologist to check for cancer cells. Special tests on the tissue will be done. This is the only way to know for sure that the changes are because of CTCL.

You may also need one or more of these tests:

• Blood tests. These can help figure out the type of CTCL and extent of the lymphoma.

• Imaging tests. These include X-rays, ultrasound, and CT, and MRI scans. They can help find out if the cancer has spread to lymph nodes or other organs.

• Other tests. You may have samples of lymph nodes and bone marrow taken to look for lymphoma cells. These tests help to figure out the stage of the disease.

After a diagnosis of CTCL, these tests help your healthcare providers learn more about your cancer. They can help figure out the stage of the cancer. The stage is how much, where, and how far the cancer has spread (metastasized) in your body. It’s one of the most important things to know when deciding how to treat the cancer.

Once your cancer is staged, your healthcare provider will talk with you about what the stage means for your treatment. Ask your healthcare provider to explain the stage of your cancer to you in a way you can understand. Early stage CTCL may be diagnosed and treated by a dermatologist. If your CTCL is advanced, you likely will be referred to a blood disease and cancer specialist (hematologist/oncologist) in CTCL, sometimes at academic centers or major hospitals where they have experience with this rare cancer.

How is cutaneous T-cell lymphoma treated? 

Your treatment choices depend on the type of CTCL you have, test results, the amount of skin that’s affected, and the stage of the cancer. The goal of treatment may be to cure you, control the cancer, or help ease problems caused by the cancer. Talk with your healthcare team about your treatment choices, the goals of treatment, and what the risks and side effects may be.

Types of treatment for cancer are either local or systemic:

• Local treatments. These remove, destroy, or control cancer cells in one area. Surgery and radiation therapy are local treatments.

• Systemic treatments. These are used to destroy or control cancer cells that may have traveled all over your body. When taken by pill or injection, chemotherapy, targeted therapy and immunotherapy are systemic treatments.

You may have just one type of treatment or a combination of treatments. 

Treatment for CTCL may include:

• Chemotherapy. Medicines to kill cancer cells may be put on the skin as a cream or gel. Or you may take them by mouth (orally) or as a shot (injection) into a vein so they can reach cancer cells all over the body.

• Other types of medicine. These may include retinoids, corticosteroids, targeted medicine, or immunotherapy. Some of these are put on the skin. Others are taken by mouth or given as a shot.

• Radiation therapy. Radiation from sources such as electrons, X-rays, and protons can be used to kill cancer cells and shrink tumors. Total skin electron beam therapy may be used to treat CTCL.

• Photodynamic therapy. This uses certain types of UV (ultraviolet) light and oral medicines called psoralens to kill cancer cells.

• Extracorporeal photopheresis. UV light and a special medicine is used to kill lymphoma cells in the blood.

Talk with your healthcare providers about your treatment choices. Make a list of questions. Think about the benefits and possible side effects of each choice. Talk about your concerns with your healthcare provider before making a decision.

Coping with cutaneous T-cell lymphoma

Many people feel worried, depressed, and stressed when dealing with cancer. Getting treatment for cancer can be hard on the mind and body. Keep talking with your healthcare team about any problems or concerns you have. Work together to ease the effect of cancer and its symptoms on your daily life.

Here are tips:

• Talk with your family or friends.

• Ask your healthcare team or social worker for help.

• Speak with a counselor.

• Talk with a spiritual advisor, such as a minister or rabbi.

• Ask your healthcare team about medicines for depression or anxiety.

• Keep socially active.

• Join a cancer support group in person or online.

Cancer treatment is also hard on the body. To help yourself stay healthier, try to:

• Eat a healthy diet, with a focus on high-protein foods.

• Drink plenty of water, fruit juices, and other liquids.

• Keep physically active.

• Rest as much as needed.

• Talk with your healthcare team about ways to manage treatment side effects.

• Take your medicines as directed by your team.

When should I call my healthcare provider?

Call your healthcare provider right away if you have any of these:

• New symptoms or symptoms that get worse

• Signs of an infection, such as a fever

Key points about cutaneous T-cell lymphoma

• Cutaneous T-cell lymphoma is a type of cancer that starts in the T-lymphocytes in the skin.

• T-lymphocytes are white blood cells that are part of the immune system.

• Older people and those who have a weakened immune system may be more at risk for this type of cancer.

• The most common symptom is dry, red, itchy, scaly rashes or patches on the skin.

• This cancer can spread from the skin into the blood, lymph nodes, and other organs.

• Treatment may include topical skin treatment, such as corticosteroids, chemotherapy, radiation therapy, and photodynamic therapy.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions, especially after office hours or on weekends.