Cutaneous T-Cell Lymphoma

CUTANEOUS T-CELL LYMPHOMA: OVERVIEW

What exactly is cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma (CTCL) is a rare cancer.

It begins in a type of white blood cell called the T-lymphocyte (T-cell). T-cells help prevent infections and other diseases.

As odd as it sounds, most T-cells are found in our skin. That’s because our skin is the first line of defense against disease. The surface of an adult’s skin contains about 20 billion T-cells. That’s nearly twice as many T-cells as found in other parts of the body.

There are many types of CTCL. More than half the people who develop CTCL will have one of the following types:

• Mycosis (my-co-sis) fungoides (fun-goi-dees)
• Sézary (sez-ah-ree) syndrome

Mycosis fungoides is the most common type of CTCL. This type tends to worsen very slowly. It can stay in its earliest stage, which often looks like rash, for years. In this stage, the cancer is often difficult to diagnose because it tends to looks like eczema or psoriasis. These conditions are much more common than CTCL.

Sézary syndrome is more aggressive. It can also look like eczema. Some people develop red and swollen skin over much of their body. Their skin may feel hot, sore, and extremely itchy.

The other types of CTCL are very rare.

Because CTCL is rare and often looks like eczema or another common skin disease, it can take time to get the diagnosis. Some people who have CTCL are referred to a dermatologist because it’s thought that they have a stubborn case of eczema or psoriasis.

If you are diagnosed with CTCL, you may receive care from a team of doctors and other health care specialists. This team may include a dermatologist, hematologist (doctor who diagnoses and treats blood diseases), oncologist (cancer doctor), and radiation oncologist (cancer doctor who specializes in using radiation to treat cancer).

Due to recent advances in treatment, many people diagnosed with CTCL live normal lives.

With this type of cancer, what you see on the skin (signs) and what you feel (symptoms) tend to vary with:

• The type of cutaneous T-cell lymphoma (CTCL)
• How far the cancer has spread (stage)

Itch: A common symptom

Although signs and symptoms tend to vary, itch can be a problem for anyone who has CTCL. It’s is often the first symptom. Studies show that between 66% and 88% of people who have CTCL develop itchy skin.

Itchy skin tends to be more common if you have:

• A more advanced stage of CTCL
• Sézary syndrome

The following describes other signs and symptoms that you may experience.

Mycosis fungoides

This is the most common type of CTCL, and it tends to grow very slowly. The first sign is often a rash or scaly patch of skin, which can look the same for years or decades.

			Earliest stage of mycosis fungoides In its earliest form, mycosis fungoides often looks like a red rash (or scaly patch of skin). It begins on skin that gets little sun, such as the upper thigh, buttocks, back, belly, groin, chest, or breasts.
			Patch stage of mycosis fungoides In this stage, you may see one or a few flat, scaly patches. This sign can also last for years, causing it to be mistaken for eczema, psoriasis, or another common skin disease.
			Patch stage of mycosis fungoides (light spots) Instead of developing scaly patches, some people develop light spots on their skin. This is more common in children, teenagers, and people who have skin of color.
			Plaque stage of mycosis fungoides As the cancer spreads, raised and often itchy patches of thicker skin appear. The medical name for these raised patches is plaques.
			Patches, plaques, and tumors on the skin As the cancer worsens, a patient may have patches, plaques, and tumors, as shown here. Some patients only have tumors. If the tumors split open, they can look like sores.

  Sézary syndrome

This type of CTCL is more aggressive than mycosis fungoides. When someone has Sézary syndrome, cancer cells are found in the skin and blood.

Not everyone who has Sézary syndrome develops widespread redness. In one study, about 75% of the patients who had Sézary syndrome developed patches on their skin that looked like eczema. These patients had thick, scaly patches of itchy skin, but their skin wasn’t red and swollen as shown in this picture.

When someone has Sézary syndrome, you may notice one or more of the following on the skin:

• Widespread redness, often with severely itchy skin
• Peeling skin
• Swollen skin
• Thickening skin on the palms, soles, or both

People who have Sézary syndrome can develop one or more of these signs:

• Swollen lymph nodes (often in the neck, armpits, and groin)
• Hair loss
• Thickened fingernails, toenails, or both
• Droopy eyelids
• Hypothermia (inability to control body temperature)

When to see a board-certified dermatologist

Many of the signs and symptoms described here occur in people who have other conditions, such as eczema. If you have been diagnosed with eczema and treatment doesn’t help, make an appointment to see a board-certified dermatologist.

Who gets cutaneous T-cell lymphoma?

The risk of developing this rare cancer increases with age. Most people are diagnosed when they are in their 50s or older. If you are African American and live in the United States, you may be diagnosed with cutaneous T-cell lymphoma (CTCL) in your 40s.

CTCL is very rare in children and teenagers.

Records show that more people are developing CTCL. This increase has been highest in men and African Americans.

What causes cutaneous T-cell lymphoma?

This cancer begins when white blood cells called T-lymphocytes (T-cells) mutate and turn into cancer cells.

Scientists still do not understand what causes these cells to mutate. They’ve studied the effects that many things, including different bacteria, genes, and chemicals can have on T-cells.

In one study, researchers discovered that the bacterium S aureus, which lives on our skin, may play a role. In this study, patients with mycosis fungoides or Sézary syndrome had very high counts of S aureus on their skin. Taking an antibiotic decreased the bacteria along with the redness and swelling on their skin.

It’s also been suggested that working with glass, pottery, or ceramics may increase the risk of developing CTCL, but this has yet to be proven.

Researchers continue to look for what causes this rare cancer.

How is cutaneous T-cell lymphoma (CTCL) diagnosed?

It can take time to diagnose cutaneous T-cell lymphoma (CTCL). This cancer is rare. If you have early CTCL, it can be difficult to find because:

• CTCL often looks like a common skin condition, such as eczema or psoriasis.
• A skin biopsy (the test for skin cancer) often doesn’t show cancer cells.
• Blood tests may not show cancer cells.

For these reasons, it’s important to keep all of your dermatology appointments. It’s important to tell your dermatologist about changes to your skin.

If your dermatologist suspects that you may have CTCL, your dermatologist will check for this during every visit by examining your skin and checking for large or swollen lymph nodes.

Your dermatologist may perform several skin biopsies. During a skin biopsy, your dermatologist removes a bit of skin so that it can be examined under a microscope.

You may also be referred to another doctor, such as an oncologist (cancer specialist) or hematologist (blood specialist), who can examine you and run tests.

CTCL is often found when doctors compare all of your test results, including those from skin biopsies and blood tests.

Because CTCL is often diagnosed by comparing the results from many tests, it can take time to get diagnosed. In a study that looked at how long it takes to diagnose CTCL, many patients were diagnosed after being tested for 3 or 4 years. Sometimes, it can take longer to diagnose CTCL.

If you are diagnosed with CTCL, you will be told the:

• Type of CTCL you have
• Stage (how far the cancer has spread)

The stages for CTCL are complex. Stage 4 is the most advanced stage. In stage 4, the cancer has spread to an internal organ.

How is cutaneous T-cell lymphoma (CTCL) treated?

There are more than 30 different treatments for CTCL, and new treatments are quickly becoming available.

In the earliest stages, treatment may be given by your dermatologist. It’s also possible that you will have a team of health care professionals who will treat you. Your team may include a dermatologist, hematologist, oncologist, primary care doctor, and nurses.

The different treatments for CTCL include the following:

Treatment for the skin

To destroy cancer cells found in the skin, you may apply medication to your skin, have radiation therapy, or get light treatments. Some patients receive a combination of treatments.

Treatments that work on the skin to treat CTCL include:

• • Mechlorethamine (me-klor-ETH-a-meen) gel: This is chemotherapy that you apply to your skin. It can slow or stop the growing cancer cells. The U.S. Food and Drug Administration (FDA) has approved it to treat the most common type of CTCL, which is mycosis (my-co-sis) fungoides (fun-goi-des), in its earliest form. For years, this drug was called nitrogen mustard gas, and some people still refer to it by this name.

• • Corticosteroid: This is not used to treat the cancer. When applied to the skin, this medication helps to reduce the redness and swelling. It can also decrease the itch and pain. Due to possible side effects, it is prescribed to treat small areas of skin.

• • Retinoid: Bexarotene (bex-air-ah-teen) gel and tazarotene (tah-zare-oh-teen) gel are retinoids used to treat early CTCL. These medications can stop or slow the growing cancer cells.

• • Radiation therapy: Doses of radiation can be quite effective at clearing CTCL on the skin. Radiation can be prescribed to treat only certain areas of the skin or the entire body. One type, electron beam radiation therapy, sends radiation to only the outer layers of the skin.

• • Light therapy: This treatment uses ultraviolet (UV) rays to kill the cancer cells in the skin. One type of light therapy is called PUVA. It involves getting a medicine called psoralen (sore-ah-lin) before being exposed to UVA light. The psoralen makes the T-cells in the skin more sensitive to light, so these cells are more easily damaged.
  • Imiquimod (eh-mick-qui-mod): This can be effective in treating early-stage CTCL. In studies, it has cleared CTCL on the skin.

Treatment that works throughout the body

Used to treat advanced CTCL, these medications include:

• • Retinoid: Acitretin (as-eh-tree-tin), bexarotene, and isotretinoin (eye-so-tre-tin-oh-in) are retinoids that you take. They have been used to successfully treat CTCL.

• • Interferon: Our bodies naturally produce interferon, which stimulates the immune system. When used to treat CTCL, interferon helps stimulate the body’s immune system so that it can attack the cancer cells. Patients may need several injections of interferon a week. Interferon may be prescribed along with another treatment for CTCL.

• • Chemotherapy: This treatment uses medication to kill cancer cells throughout the body. Healthy cells that grow quickly, such as hair cells, may also be damaged by chemotherapy. Several medications, including brentuximab vedotin (bren-tux-eh-mab vee-dot-in) and gemcitabine (gem-seh-tab-ean) are used to treat CTCL.

• • Extracorporeal photopheresis (ECP): When cancer cells are found in the blood, this may be a treatment option. ECP involves removing blood with a catheter, treating the cancer cells, and then returning the treated blood to your body. While the blood is out of your body, the white blood cells are treated with UV light, which damages the cancer cells. To receive this treatment you would go to a cancer center or hospital.

• • Targeted therapy: These medications help stop the cancer cells from growing and spreading. Unlike chemotherapy medications that destroy entire cells, this treatment targets only specific parts of the cancer cells. This causes fewer side effects than chemotherapy. Targeted therapies for CTCL include vorinostat (vor-in-oh-stat) and romidepsin (row-meh-dep-sin).

• • Stem cell transplant: This is a newer option for patients who have CTCL, so less is known about it. In one study, researchers followed 47 patients who had a stem cell transplant after other treatments for CTCL failed to work. After 4 years, about half the patients who received a stem cell transplant were alive and 26% still had the CTCL but it hadn’t gotten worse. If this is an option for you, you will need someone, such as a sibling, to donate healthy stem cells. The healthy stem cells are given after you receive chemotherapy to destroy as many cancer cells as possible.
  • Clinical trial: Many new treatments for CTCL are being studied in clinical trials. This may be an option for you if other treatments haven’t worked or you are a good candidate for a treatment being studied.

Patients receive follow-up care

Patients diagnosed and treated for CTCL receive follow-up care. What’s involved in your follow-up care will depend on whether you have finished treatment or will need to continue treatment.

During follow-up visits, your doctor will examine you closely, looking for new signs of CTCL. You may also need blood tests and other medical testing.

What is the outcome for someone who has cutaneous T-cell lymphoma?

The most common type of cutaneous T-cell lymphoma (CTCL), mycosis fungoides, grows slowly. For this reason, about 70% of patients have early-stage cancer when diagnosed. When treatment begins in the early stages, a person has a normal life expectancy.

When the cancer is more advanced, the goals of treatment are to reduce the tumors, delay the spread of the cancer, and preserve a person’s quality of life. While CTCL cannot be cured, advances in treating this cancer are making these goals possible for more patients.

Today, many people who have CTCL die of another cause.

Images used with permission of the Journal of the American Academy of Dermatology.