Cutaneous T-cell lymphoma

What is cutaneous t-cell lymphoma?

Definition: Any of a group of T-cell non-Hodgkin lymphomas that begins in the skin as an itchy, red rash that can thicken or form a tumor. The most common types are mycosis fungoides and Sézary syndrome.

CTCL (cutaneous T-cell lymphoma) is an uncommon type of cancer that starts in T cells, which are white blood cells (T lymphocytes). These cells generally assist your body’s immune system in battling germs. The T cells in cutaneous T-cell lymphoma develop defects that cause them to assault the skin.

Cutaneous T-cell lymphoma causes rash-like skin redness, slightly raised or scaly circular spots on the skin, and skin tumours in some cases.

There are several forms of cutaneous T-cell lymphoma. Mycosis fungoides is the most frequent form. Sezary syndrome is a rarer type of skin redness that affects the entire body. Some forms of cutaneous T-cell lymphoma, such as mycosis fungoides, proceed slowly while others progress quickly.

Which therapy are best for you depends on the type of cutaneous T-cell lymphoma you have. Skin creams, light therapy, radiation therapy, and systemic drugs like chemotherapy are all options for treatment.

Non-lymphoma Hodgkin’s refers to a group of lymphomas that include cutaneous T-cell lymphoma.

Types of cutaneous T-cell lymphoma (CTCL)

CTCL describes many different disorders with various symptoms, outcomes, and treatment considerations:

• Mycosis Fungoides (MF): the most common type of CTCL, accounting for approximately one-half of all CTCLs. MF can look different in each patient, with skin symptoms that can appear as patches, plaques, or tumors.
• Sézary Syndrome: characterized by the presence of lymphoma cells in the blood, patients with Sézary Syndrome often present with extensive thin, red, itchy rashes that typically appear on the skin.

Symptoms of cutaneous T-cell lymphoma

Signs and symptoms of cutaneous T-cell lymphoma include:

• Round patches of skin that may be raised or scaly and might be itchy
• Patches of skin that appear lighter in color than surrounding skin
• Lumps that form on the skin and may break open
• Enlarged lymph nodes
• Hair loss
• Thickening of the skin on the palms of the hands and soles of the feet
• A rash-like skin redness over the entire body that is intensely itchy

Causes of cutaneous T-cell lymphoma

It’s unclear what causes cutaneous T-cell lymphoma.

In general, cancer develops when cells’ DNA undergoes alterations (mutations). The DNA of a cell carries instructions that tell it what to do. The DNA mutations instruct the cells to expand and multiply quickly, resulting in a large number of aberrant cells.

Mutations in cutaneous T-cell lymphoma cause an overabundance of aberrant T cells that attack the skin. T cells are immune cells that ordinarily assist your body in fighting pathogens. Doctors are baffled as to why the cells are attacking the skin.

Diagnosis of cutaneous T-cell lymphoma

Tests and procedures used to diagnose cutaneous T-cell lymphoma include:

• Physical exam. Your doctor will examine your skin for patchy, scaly regions or solid, raised growths. You will also be examined for signs that your lymph nodes or other organs might be affected.
• Blood tests. Blood tests such as the complete blood count might be used to better understand your condition. Sometimes cancer cells are found in the blood, particularly with Sezary syndrome.

• Skin biopsies. A procedure to cut away a small sample of skin (skin biopsy) is usually needed to diagnose cutaneous T-cell lymphoma. The skin might be cut with a circular tool (punch biopsy). For larger lesions and tumors the biopsy might be done with a small knife (excisional biopsy).

  A doctor who specializes in analyzing blood and tissue (pathologist) examines the sample in a lab to determine whether it contains cancer cells. Sometimes multiple skin biopsies are necessary to confirm your diagnosis. Advanced lab tests to analyze the tissue might uncover clues about your cancer that will help your doctor understand your prognosis and determine your treatment options.

• Imaging tests. If there’s concern that the cancer cells have spread to other parts of the body, your doctor might recommend imaging tests, such as computerized tomography (CT) or positron emission tomography (PET) scans.

Treatment of cutaneous T-cell lymphoma

People with cutaneous T-cell lymphoma can choose from a variety of treatments. The optimal treatment for you is determined by your unique circumstances, including the amount and stage of your lymphoma. The majority of persons with cutaneous T-cell lymphoma undergo a combination of therapies.

Treatment options may include:

• Skin creams and ointments. Medicines can be applied to your skin in the form of creams, gels and ointments. Corticosteroids can help control skin redness and itchiness. Chemotherapy can be applied to the skin to attack cancer cells.
• Light therapy (phototherapy). Phototherapy involves exposing the skin to wavelengths of light, such as ultraviolet B or ultraviolet A. Various machines are used in phototherapy, including booths that expose most of your body to the light. Sometimes phototherapy is done after the application of a medication that makes skin cells more sensitive to light (photodynamic therapy). Healthy cells regenerate quickly, but cancer cells do not.
• Radiation therapy. Radiation therapy uses beams of energy to kill cancer cells. If you have one area of cutaneous T-cell lymphoma, standard radiation therapy with X-rays might be recommended. For people with more areas of cancer, radiation therapy might be done with electron beams, which target the skin and don’t affect internal organs. Electron beam radiation is usually applied to all of the skin.
• Medications. Medications used to treat cutaneous T-cell lymphoma include treatments to control the immune system, such as steroid drugs and interferon. Chemotherapy medicines attack quickly growing cells, including cancer cells. Targeted therapy medicines attack cancer cells by targeting the cells’ specific vulnerabilities.
• Exposing blood cells to light. A procedure called extracorporeal photopheresis involves taking a medicine that makes your cells more sensitive to light. Then your blood is filtered through a machine that exposes it to ultraviolet light before returning the blood to your body.
• Bone marrow transplant. A bone marrow transplant, also known as a stem cell transplant, is a procedure to replace your diseased bone marrow with healthy bone marrow from a matched donor (allogeneic bone marrow transplant). During a transplant you’ll receive chemotherapy drugs to suppress your diseased bone marrow. Then healthy donor cells are infused into your body where they travel to your bones and begin rebuilding your bone marrow.