Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) is an uncommon type of non-Hodgkin lymphoma (NHL). CTCL is a cancer that starts in mature T cells in the skin. Other types of B-cell and T-cell lymphoma can affect the skin, but they are different from CTCL.

CTCL usually occurs in adults who are 50 to 60 years old. It develops more often in men than in women.

CTCL is not one disease. It is a group of different T-cell lymphomas that affect the skin.

Mycosis fungoides is the most common form of CTCL. It starts in the skin on areas of the body that are not usually exposed to the sun. It may appear as a scaly, itchy red rash or raised lumps (called plaques). Mycosis fungoides may be hard to diagnose because it can be confused with other skin problems. It is slow growing (indolent), and the symptoms may be present for many months or years before mycosis fungoides is diagnosed. The disease can progress to tumours on the skin and may eventually spread to the lymph nodes, liver, spleen, lungs or blood. In rare cases, it can spread to the bone marrow.

Sezary syndrome is very rare. Doctors used to think of it as an advanced form of mycosis fungoides, but it is actually a different disease. Sezary syndrome is fast growing (aggressive). The lymphoma cells are in the skin, blood and lymph nodes. Most or all of the skin on the body is affected. It can look like a sunburn, and the skin is often red, itchy and peeling. In advanced stages the skin may be very itchy, painful and intolerant to cold. The disease may also spread to other organs and cause other symptoms. People with Sezary syndrome often have weakened immune systems and a higher risk for infections.

Primary cutaneous anaplastic large cell lymphoma (ALCL) is slow growing (indolent). It usually starts as only one lump or a few lumps or tumours in the skin. The tumours can be different sizes and some may break open and become an open sore. Primary cutaneous ALCL is usually only found in the skin. It may spread to nearby lymph nodes. It rarely spreads to other organs. Primary cutaneous ALCL is different from systemic ALCL that affects the skin.

CTCL can cause different types of skin problems. In the early stages, the tumours on the skin may be mistaken for non-cancerous conditions like psoriasis or eczema. CTCL can cause:

• small lumps that look like pimples (called papules)
• flat, thin, red patches
• thick, raised areas (called plaques)
• lumps that can be felt (called nodules or tumours)
• lumps with open sores

Different treatments can be used for CTCL. Some treatments are localized, which means they are given only to the affected skin. Other treatments are systemic, which means they travel through the whole body.

Ultraviolet light therapy uses either ultraviolet A (UVA) or ultraviolet B (UVB) light to help destroy lymphoma cells. It is given with a special type of light.

PUVA combines psoralen and UVA light. Psoralen is a drug taken as a pill by mouth. It is usually given about 2 hours before the skin is treated with the UVA light. Psoralen makes the skin very sensitive to the effects of UVA light, which helps destroy the lymphoma cells. PUVA treatments are given about 3 times a week until there are no more signs of CTCL on the skin. They are continued less often as maintenance therapy.

UVB phototherapy uses only UVB light because you don’t need to take a drug to make the skin sensitive to it. It is often used to treat thin patches of CTCL. UVB phototherapy is usually given 3–4 times a week until there are no more signs of CTCL on the skin (about 30–40 treatments). Then it is given less often as maintenance therapy.

External beam radiation therapy may be used alone or with other treatments for CTCL. Electron beam radiation therapy is commonly used. It uses electrons to deliver the radiation. The electrons don’t go all the way through the skin so the radiation doesn’t affect other organs or the bone marrow.

Local external beam radiation therapy means the radiation is given to a small area of skin. It may be used if there is only one tumour or if there are thicker tumours in one small area. It may also be used to treat patches that remain after PUVA.

Total skin electron beam (TSEB) therapy means that radiation is given to skin all over the body. It is used if CTCL is more widespread. It is usually given only once, but it can sometimes be repeated using lower doses if CTCL comes back after treatment. TSEB therapy can cause a sunburn-like reaction, and some people may lose their fingernails, toenails and hair.

Chemotherapy drugs used to treat CTCL are put directly onto the skin (called topical chemotherapy) as an ointment or liquid. They can also be given as a systemic therapy that circulates through the blood and destroys cancer cells all over the body.

Topical chemotherapy is usually given to treat early stage CTCL that only affects small areas of the skin (called limited disease). The topical chemotherapy drugs that may be used include:

• mechlorethamine (nitrogen mustard, Mustargen)
• carmustine (BiCNU, BCNU)

Systemic chemotherapy is used to treat CTCL that is more advanced and has spread to other organs. It is also used to treat CTCL that comes back after (recurs) or that no longer responds to other treatments. Chemotherapy drugs used to treat CTCL include:

• fludarabine (Fludara)
• cladribine (Leustatin)
• pentostatin (deoxycoformycin, Nipent)
• pegylated liposomal doxorubicin (Caelyx)
• gemcitabine (Gemzar)
• methotrexate
• chlorambucil (Leukeran)
• etoposide (Vepesid, VP-16)
• temozolomide (Temodal)
• pralatrexate (Folotyn)
• vorinostat (Zolinza)

The chemotherapy combinations that may be used to treat CTCL are:

• CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone
• R-CHOP – CHOP with rituximab (Rituxan)
• EPOCH – etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone

Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunotherapy drugs like interferon alfa (Intron A, Wellferon) may be used to help boost the immune response and treat CTCL. Interferon alfa is injected into fat underneath the skin using a needle. It may be used alone or with other treatments, such as PUVA.

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells. Targeted therapy drugs used to treat CTCL include:

• rituximab (Rituxan)
• alemtuzumab (Campath)
• denileukin diftitox (Ontak), which is a combination of interleukin-2 (Aldesleukin, Proleukin) and the diphtheria toxin
• romidepsin (Istodax)
• brentuximab vedotin (Adcetris)

Photopheresis is also called extracorporeal photopheresis, or ECP. It is a systemic treatment that is sometimes used to treat Sezary syndrome or CTCL that is progressing.

The procedure for photopheresis is similar to donating blood, but the blood goes into a special machine rather than a collection bag. The machine takes out the lymphocytes, including the lymphoma cells. The lymphocytes are treated with psoralen and UVA light. After they are treated, the lymphocytes are mixed with the rest of the blood and given back to the person. The procedure takes a few hours. It is usually done for 2 days in a row. It is then repeated about every 4 weeks.

Surgery is sometimes used for CTCL. It is most often used to do a skin biopsy to diagnose CTCL. In some cases, it is used to completely remove tumours on the skin. Even if surgery is used to remove a tumour, other treatments will also be used.

A stem cell transplant is sometimes offered for CTCL that doesn’t respond to other treatments.