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Cutaneous T-cell lymphoma: Diagnosis and treatment


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.

Medications and supplements

Be sure your doctors know all of the medications and supplements you take. Some can prevent treatment for CTCL from working.

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.

    Be sure to tell your doctore if you have diabetes

    If a retinoid is prescribed, be sure to tell your doctor if you have diabetes. Retinoids can affect your sugar levels, so you’ll have to watch your levels carefully.

  • 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 and romidepsin.

  • 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 four 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
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References
Bagherani N and Smoller BR. “An overview of cutaneous T cell lymphomas.” F1000Res. 2016; 5.

Horwitz SM, Ansell S, et al. “National Comprehensive Cancer Network: Clinical practice guidelines in oncology: T-cell lymphomas.” Version 3.2018. Last accessed 2.23.2018.

Hosing C, Bassett R, et al. “Allogeneic stem-cell transplantation in patients with cutaneous lymphoma: updated results from a single institution.” Ann Oncol. 2015; 26(12):2490-5

Jawed, BA, Myskowski PL, et al. “Primary cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome) Part I. Diagnosis: Clinical and histopathologic features and new molecular and biologic markers.”  J Am Acad Dermatol 2014; 70:205.e1-16.

Jawed, BA, Myskowski PL, et al. “Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.” J Am Acad Dermatol. 2014; 70(2):223.e1-17.

Martin AG. “Bexarotene gel: a new skin-directed treatment option for cutaneous T-cell lymphomas.” J Drugs Dermatol. 2003; 2(2):155-67.

Martínez-González MC, Verea-Hernando MM, et al. “Imiquimod in mycosis fungoides.” Eur J Dermatol. 2008;18(2):148-52.

Wilcox RA. “Cutaneous T-cell lymphoma: 2017 update on diagnosis, risk-stratification, and management.” Am J Hematol. 2017; 92(10):1085-1102.

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