Cutaneous T-Cell Lymphoma (CTCL)
Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells).
T-cell lymphomas account for approximately 15 percent of all NHLs in the United States. There are many different forms of T-cell lymphomas, some of which are extremely rare. Most T-cell lymphomas can be classified into two broad categories: aggressive (fast-growing) or indolent (slow-growing).
One of the most common forms of T-cell lymphoma is cutaneous T-cell lymphoma (CTCL), a general term for T-cell lymphomas that involve the skin. CTCL also can involve the blood, the lymph nodes, and other internal organs. Symptoms can include dry skin, itching (which can be severe), a red rash, and enlarged lymph nodes. The disease affects men more often than women and usually occurs in men in their 50s and 60s.
Most patients with CTCL experience only skin symptoms, without serious complications; however, approximately 10 percent of those who progress to later stages develop serious complications. Early stage CTCL is typically indolent; some patients with early-stage CTCL might not progress to later stages at all, while others might progress rapidly, with the cancer spreading to lymph nodes and/or internal organs.
CTCL describes many different disorders with various symptoms, outcomes, and treatment considerations. The two most common types are mycosis fungoides and Sézary syndrome.
Mycosis fungoides is the most common type of CTCL, with approximately 16,000 to 20,000 cases across the United States, accounting for half of all CTCLs. The disease looks different in each patient, with skin symptoms that can appear as patches, plaques, or tumors. Patches are usually flat, possibly scaly, and look like a rash; plaques are thicker, raised, usually itchy lesions that are often mistaken for eczema, psoriasis, or dermatitis; and tumors are raised bumps, which may or may not ulcerate. It is possible to have more than one type of lesion.
A medical history, physical exam, and skin biopsy are essential for diagnosis. A physician will examine lymph nodes, order various blood tests, and may conduct other screening tests, such as a chest x-ray or a computed axial tomography (CAT) scan. Scans are usually not needed for those with the earliest stages of the disease.
Mycosis fungoides is difficult to diagnose in its early stages because the symptoms and skin biopsy findings are similar to those of other skin conditions.
Sezary syndrome is an advanced, variant form of mycosis fungoides, which is characterized by the presence of lymphoma cells in the blood. Extensive thin, red, itchy rashes usually cover over 80 percent of the body. In certain patients, patches and tumors appear. Patients may also experience changes in the nails, hair, or eyelids, or have enlarged lymph nodes.
Many of the same procedures used to diagnose and stage other types of cutaneous T-cell lymphomas are used in Sézary syndrome. In addition, a series of imaging tests may be needed to determine if the cancer has spread to the lymph nodes or other organs (although that uncommonly occurs). These tests may include a CAT scan, a positron emission tomography (PET) scan, and/or a magnetic resonance imaging (MRI) scan. A bone marrow biopsy may also be done, but is usually not necessary.
Treatment selection for CTCL depends on the extent of skin involvement, the type of skin lesion, and whether the cancer has spread to the lymph nodes or other internal organs.
For mycosis fungoides, treatment is either directed at the skin or the entire body (systemic). Because Sézary syndrome is chronic and systemic (affecting the entire body), it is usually not treated with skin-directed therapies alone. Treatments may be prescribed alone or in combination to achieve the best long-term benefit. Many patients live normal lives while they treat their disease, and some are able to remain in remission for long periods of time.
Skin-directed therapies are useful for patch and limited plaque disease and include topical treatments such as corticosteroids, retinoids, or imiquimod (which activates immune cells), topical chemotherapy, local radiation, methotrexate, photopheresis, ultraviolet light (phototherapy).
On August 23, 2013, the U.S. Food and Drug Administration (FDA) approved mechlorethamine gel (Valchlor) for the topical treatment of patients with mycosis fungoides, a type of cutaneous T-cell lymphoma (CTCL), who have received prior skin-directed therapy. This represents the first and only FDA-approved topical formulation of mechlorethamine. This treatment was previously approved as an intravenous treatment for the same disease.
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Systemic therapies for more advanced disease are usually deferred until patients have not responded well to topical therapies. More advanced disease is commonly treated with radiation, chemotherapy, and/or therapies such as:
Combination chemotherapy regimens are generally reserved for when patients have not responded well to several single-agent therapies. Options for refractory disease (disease that no longer responds to the initial therapy) include alemtuzumab (Campath), liposomal doxorubicin (Doxil), and gemcitabine (Gemzar).
Patients in remission should have regular visits with a physician who is familiar with their medical history as well as with the treatments they have received. Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.
A lymphoma diagnosis often triggers a range of feelings and raises many concerns. In addition, cancer treatment can cause physical discomfort. Support groups and online message boards can help patients connect with other people who have lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network, match lymphoma survivors (or caregivers) with volunteers who have gone through similar experiences.
Lymphoma survivors should receive regular medical exams from a physician who is familiar with their medical history as well as the treatments they have received. Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences.
LRF T-Cell Lymphoma Transportation Assistance Fund
The Cutaneous Lymphoma Foundation is an independent nonprofit patient advocacy organization dedicated to supporting every person with cutaneous lymphoma by promoting awareness and education, advancing patient care, and facilitating research. To find out more information about the resources that the Cutaneous Lymphoma Foundation provides, please visit their website (www.clfoundation.org).Webcasts/Podcasts
• Update on T-Cell Lymphoma - September 17, 2014
In-person Educational Programs
View or order the following publications from our booklets/factsheets webpage:
Professional staff members are available to answer your questions and provide individual support to you and your loved ones. Contact our Helpline, available Monday through Friday from 8:00am - 5:00pm Pacific Standard Time (PST). Call (800) 500-9976 or e-mail.