Overview
Cutaneous T-cell lymphoma is a rare type of lymphoma that mainly affects the skin. Lymphoma is a cancer that affects the lymphocytes, which are a type of white blood cell. The white blood cells are part of the body's germ-fighting immune system.
There are many types of lymphoma. These types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. Cutaneous T-cell lymphoma (CTCL), is a type of non-Hodgkin lymphoma.
CTCL gets its name from the kind of cell that makes up this cancer. It begins in white blood cells called T cells. These cells also are called T lymphocytes. The T cells undergo changes and turn into cancer cells. A cell's DNA holds the instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells don't die. They usually multiply and form a mass. In cutaneous T-cell lymphoma, the T cells attack the skin.
Cutaneous T-cell lymphoma is not a single condition. There are several types of CTCL. Depending on the type you have, it can appear on the skin as a rash or as slightly raised or scaly patches in various shapes, sizes and colors. Sometimes other growths, including firm bumps and tumors, may appear within the skin.
CTCL is often hard to diagnose early because it usually looks like the skin conditions eczema, and psoriasis when it first starts. The rash can go away and come back. This cycle can repeat for months or years before the rash becomes worse or the prescribed treatment stops working. CTCL also is easy to miss in tests when it first appears. Under the microscope, the cancer cells may look like cells from a typical rash. Sometimes the skin has to be studied many times before a diagnosis is reached.
Cutaneous T-cell lymphoma typically cannot be cured. But many treatments can help reduce symptoms, improve the skin appearance and slow the cancer's growth. Treatment depends on the type of CTCL you have. If the cancer is only in the skin and in early stages, healthcare professionals may start with skin-directed treatments. If the cancer forms tumors or spreads to the blood, lymph nodes or organs, systemic treatments, which work throughout the whole body, usually are given.
Skin-directed treatments may include skin creams, light therapy and surface-level radiation therapy. Chemotherapy is a systemic treatment. A combination of several of these options may be used. These treatments help manage the disease and improve quality of life.
Dr. Rafael Fonseca, hematologist and Chief Innovation Officer at Mayo
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Types
There are several types of cutaneous T-cell lymphoma (CTCL). Some types are indolent, which means the cells grow slowly. In other types, the cells grow quickly. The CTCL types include:
- Mycosis fungoides. Mycosis fungoides is the most common type of CTCL. It typically grows slowly and mainly looks like scaly patches similar to eczema or psoriasis. In a small number of people, it can worsen quickly.
- Sezary syndrome. Sezary syndrome grows and expands quickly. It affects the skin and the blood. In time, it typically causes a rash over the whole body.
- Subcutaneous panniculitis-like T-cell lymphoma. This type is usually slow growing. It mainly affects the fatty layer underneath the skin and may feel like a hard lump under the skin. It usually appears on the arms, legs, face and torso. The torso includes the chest, stomach and pelvic area.
- Primary cutaneous gamma-delta T-cell lymphoma. Primary cutaneous gamma-delta T-cell lymphoma is typically fast growing. It may cause thickened patches of skin, lumps, tumors or ulcers in the skin.
- Primary cutaneous anaplastic large cell lymphoma. This type usually appears on or in the skin as slow-growing lumps.
There are many types of cancer that start in the immune cells and affect the skin. People sometimes wonder if CTCL is the same as cutaneous B-cell lymphoma. CTCL is a type of non-Hodgkin lymphoma that starts in the T cells. Cutaneous B-cell lymphoma is a type of non-Hodgkin lymphoma that involves B cells, which is another type of immune system cell. People who have lymphomas involving B cells often have different treatment plans.
Symptoms
Signs and symptoms of cutaneous T-cell lymphoma (CTCL) include:
- Patches of flat, raised or scaly skin that might itch. The patches happen most often on skin that doesn't get much sun, including the upper thighs, breasts and buttocks.
- Patches of skin that look pink, red, brown or gray. They may be harder to see on Black and brown skin.
- Patches of skin that look lighter in color than the skin around them. They may be harder to see on white skin.
- Lumps that form on the skin and may break open.
- Lymph nodes that get bigger.
- Hair loss, including on the body and scalp.
- Thickened skin on the palms of the hands and soles of the feet.
- A rash over all the skin that is scaly and itchy.
When to see a doctor
Make an appointment with a healthcare professional if you have symptoms that worry you or are not going away. This might include an itchy rash that is worsening or not responding to prescribed medication.
Causes
Cutaneous T-cell lymphoma (CTCL) begins in white blood cells called T cells. T cells also are called T lymphocytes. In one type of CTCL called Sezary syndrome, the cancer cells also are in the blood.
The T cells undergo changes and turn into cancer cells. A cell's DNA holds the instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells don't die. They usually multiply and form a mass. In CTCL, the T cells attack the skin. Exactly why these DNA changes happen is not known.
Risk factors
The risk of cutaneous T-cell lymphoma (CTCL) may be higher in:
- Older adults. The condition can happen at any age, but it's more common in people age 50 and older.
- People assigned male at birth. The condition is twice as common in people assigned male at birth than it is in people assigned female at birth.
- Black people. Black people have the highest risk of getting CTCL and are more likely to get this cancer at an earlier age.
There is no known way to prevent CTCL.
Complications
Cutaneous T-cell lymphoma (CTCL) may cause a number of complications as the cancer grows and spreads to other areas of the body. Complications vary by type. For example, mycosis fungoides and Sezary syndrome may cause infections and additional types of lymphoma.