Cutaneous T-Cell Lymphoma
What is cutaneous T-cell lymphoma (CTCL)?
Lymphoma is the most frequent form of blood cancer, occurring when lymphocytes (a type of white blood cell) multiply uncontrollably. The two main kinds of lymphocytes that develop into lymphomas are B-cells and T-cells.
CTCL is a general term for T-cell lymphomas that affect the skin. There are three main subtypes:
- Mycosis fungoides: The most frequent type of CTCL (50 percent of all cases), it usually involves only the skin and grows slowly over many years.
- Sézary syndrome: The second most common type of CTCL (15 percent of all cases). Lymphoma cells are found in both the skin and the bloodstream.
- CD30-positive lymphoproliferative disorders: This type includes a wide range of CTCLs, some of which can grow very quickly.
How common is cutaneous T-cell lymphoma (CTCL)?
CTCL is a rare form of T-cell lymphoma. There are about 3,000 new cases of CTCL in the U.S. each year, and about 16,000 – 20,000 Americans have mycosis fungoides.
How does a person get cutaneous T-cell lymphoma (CTCL)?
There is currently no clearly identified cause of CTCL. It is not contagious and is not an infection.
Who is affected by cutaneous T-cell lymphoma (CTCL)?
CTCL affects males twice as often as females. It affects older people (usually in the age range of 40 to 60 years) more often than younger people. CTCL affects people of African-American heritage two times as often as those of European or Asian ancestry.
Symptoms and Causes
What are the symptoms of cutaneous T-cell lymphoma (CTCL)?
Symptoms of CTCL can differ greatly, depending on the disease type and stage. The most common type is mycosis fungoides, whose symptoms progress slowly through three separate phases.
First phase: Red rash or dry, red, scaly patches of skin usually on the buttocks and trunk. These patches may remain and not change, may go away on their own, or slowly get larger. In this phase, the skin resembles psoriasis and it may remain this way for months, years or decades.
Second phase: Raised up, reddish-brown, scaly bumps (plaques) appear. These plaques may develop out of pre-existing patches or in previously unaffected areas. Over time they may grow together and form larger plaques. Any part of the body may be affected.
Severe itchiness and pain might also be present in the first two phases, but not all people experience this.
Third phase: Development of mushroom-shaped tumors. In some cases they may break open (ulcerate) or become infected. Some people develop tumors without ever experiencing the symptoms of the first two phases.
In about 10 percent of mycosis fungoides cases, lymphocytes spread from the skin into lymph nodes and/or internal organs such as the liver, spleen or digestive system. This could present a life-threatening situation.
Sézary syndrome is a rare form of CTCL. It is marked by the presence of cancerous lymphocytes in the bloodstream, and enlarged lymph nodes. Severe itching, and thickening, scaling and peeling of the skin may be present. Other symptoms include outward-turning eyelids, malformed nails, bald patches on the head, fevers, and general fatigue.
Diagnosis and Tests
How is cutaneous T-cell lymphoma (CTCL) diagnosed?
- Review of medical history
- Physical examination with special attention to the number and type of skin lesions
- Blood tests including complete blood count with differential, peripheral blood smear, and blood flow cytometry
- Skin biopsy or sampling
- Imaging tests such as computed tomography (CT) or positron emission tomography (PET)
- Bone marrow or lymph node biopsy
Management and Treatment
How is cutaneous T-cell lymphoma (CTCL) treated?
Once a diagnosis of CTCL has been made it is important to find out if cancer has spread from the skin to any other part of the body. This process is called staging, and will determine the proper choice of treatment and long-term outlook. There are five categories of standard treatments:
- Photodynamic therapy: A drug that is activated by laser light is injected into a vein. The drug accumulates in cancer cells. When laser light is shined onto the skin, the drug kills cancer cells.
- Extracorporeal photophoresis: Another treatment that involves light. The patient’s blood is drawn. White blood cells are culled out and mixed with a drug that makes cancerous T-cells sensitive to ultraviolet (UV) light. When exposed to UV light, these cells die. The treated white blood cells are then returned to the patient.
- Radiation therapy: X-rays or other sources of radiation directed at cancer cells can kill them or slow their growth. External radiation sources include a radiation therapy machine, which can direct electrons (subatomic particles with a negative charge) or photons to diseased areas of skin.
- Chemotherapy: Chemotherapy may be given by mouth, injection, or as gels, creams or lotions spread directly onto the skin. Examples of chemotherapy drugs include methotrexate and gemcitabine.
- Other drug therapy:
- Corticosteroids are applied directly to the skin to treat red, swollen and inflamed skin.
- Retinoids such as bexarotene are vitamin A-related drugs that can slow the growth of cancer cells.
- Histone deacetylase inhibitors like vorinostat and romidepsin keep tumor cells from dividing.
- Lenalidomide provides a boost to the body’s own immune system to help kill diseased cells, and prevent the growth of new blood vessels in tumors.
- Biologic therapy: Substances made by the body or in a laboratory add to the patient’s immune system’s ability to battle disease. Interferons, for example, interfere with cancer cell division and can slow the growth of tumors.
What are the complications/side effects of treatments for cutaneous T-cell lymphoma (CTCL)?
Cancer treatments can cause side effects because healthy tissue or organs are affected along with the cancer. Common side effects include but are not limited to:
- Loss of appetite
- Bleeding and bruising
- Hair loss
- Nausea and vomiting
- Problems with sexual function and fertility
Outlook / Prognosis
What is the outlook for patients with cutaneous T-cell lymphoma (CTCL)?
There is no cure for CTCL but many patients are able to keep the disease under control and remain free of symptoms for years. Most patients receiving treatment for early-stage mycosis fungoides have a normal life expectancy.
The following factors determine how well a patient is able to manage the disease:
- The stage of the cancer
- The type and number of skin abnormalities present
- The patient’s age, sex and general health
When should I see a healthcare provider about cutaneous T-cell lymphoma (CTCL)?
Patients who are being treated for CTCL should have regular follow-up visits with a doctor who knows their medical history and course of treatment. Tests may be needed periodically to determine if additional treatments are required, and to check for side effects. The longer CTCL symptoms remain under control, the less necessary visits to the doctor will become.