Cutaneous T-Cell Lymphoma (CTCL) is a type of lymphoma, or cancer of the immune cells, that arises in T-cells in the skin (primarily). T-cells are an integral part of the immune system, helping to recognize and fight against viruses, bacteria, and even other types of cancers. CTCL is a rare form of non-Hodgkin lymphoma and is most common in the skin, but may affect the blood, lymph nodes, and other internal organs as well. This disease affects men more often than women, and usually occurs between the ages of 40 and 70. There are many forms of treatment that are used to slow the progression of CTCL, but none that serve as curative. Recently, however, there have been a number of emerging biologic and targeted therapies that can have impressive success with treating CTCL. This article will focus on the diagnosis/treatment planning of CTCL as well as describe several widely used treatments for CTCL.
|A computer-generated microscope image of T-cells|
Diagnosis & Stages of CTCL
CTCL can often be diagnosed by a dermatologist after a physical examination, blood tests, a skin biopsy, and a series of imaging tests. Patients often come to their dermatologist complaining of a scaly red rash that appears in patches on areas of the body that do not get sun exposure, a thin/reddish eczema rash, tumors on the skin, or full body skin redness with itching. One diagnostic test that is commonly used to diagnose lymphoma is known as immunophenotyping. This process detects markers on the surface of immune cells in your blood to compare cancer cells with normal cells and identify the specific type of cancer you have. Once it has been established that you have CTCL, a treatment plan must be developed. Treatment plans vary from patient to patient and depend heavily on what stage of the disease you are in.
Similar to most cancers, CTCL can be described as occurring in 4 main stages, with most of these broken down further into substages. Your doctor will be able to determine which stage you are in during your diagnosis. Stage I is broken into stage IA and IB, but both are characterized by a small portion (around 10%) of the skin surface covered with a patched rash and/or plaque. Stage II is broken into stages IIA and IIB which are characterized by any amount of the skin covered in patches or plaques and in IIB, one or more tumors on the skin surface with enlarged lymph nodes. Stage III is characterized by a condition known as erythrodermic skin which involves a diffuse pattern of red patches or plaques covering over 80% of the skin. Finally, stage IV is broken into stages IVA and IVB which are characterized by a culmination of the previous stages as well as cancer involving the lymph nodes, blood, and other organs in the body (IVB).
After reading this description of how CTCL advances through several stages, it is clear why each patient needs a unique treatment plan. Patients in later stages of the disease may elect a more aggressive treatment plan or may need to opt for a less aggressive plan to avoid injury due to a weakened state. These concerns need to be addressed through an extended conversation with your doctor about your own unique condition and how you would like to (or are able to) move forward. Once you understand your own condition the door is opened to learn more about the various treatment options available to you.
An Overview of Commonly Prescribed Treatments
There are many types of treatment options available for CTCL and related cancers. Some are applied directly to the skin (topical), while others are more general, affecting the whole body. Patients in the early stages of CTCL tend to respond more readily to topical treatments, while patients with cancers that don’t respond readily to treatment, or who are in later stages, may require more systemic treatments. The types of treatments that may be used include topical therapy, biologic or immune therapy, chemotherapy, phototherapy, and radiation therapy.
Topical therapies include corticosteroids, topical chemotherapy, and retinoids. Corticosteroids are used to reduce swelling in inflamed skin, a common symptom of CTCL. These steroids can also be used to slow the growth of new cancerous cells, as well as to eliminate affected T-cells. Topical chemotherapy involves the application of various ointments which can be used to treat stage I CTCL. Retinoids are related to vitamin A and work by boosting the immune response and helping the cancer cells to die more rapidly. Topical treatments are all very easy to apply and tend to work well for early stage CTCL.
Radiation Therapy can be used either on its own, or in combination with other types of CTCL therapy. Radiation is very versatile and can be controlled in both dosage and application to apply to organs beneath the skin, or only the skin itself. This makes radiation a useful therapy at most stages of CTCL for many patients. On top of this, radiation often causes the death of cancerous cells without leaving a scar or open injury, reducing the risk of infection and other side effects. Radiation is commonly used to treat CTCL in small doses to avoid damaging the skin or increasing the risk of skin cancer in susceptible individuals.
Biologic/Immune Therapies are mild systemic therapies that help cancerous cells to die more quickly or activate a patient’s own immune system to better fight off the cancer. There are many types of biologic and immune therapies that can be prescribed at various stages of the CTCL progression making this class of treatment quite flexible. One form of this therapy which has recently shown to be tolerated quite well by CTCL patients is extracorporeal photopheresis (ECP). During this procedure, the patient’s blood is drawn through a machine and (cancerous) white-blood cells are separated from the rest of the blood. These cells are then made susceptible to UV light and then exposed to this type of light, slowly killing the cells. When these cells are returned to your body, your immune system recognizes them as decaying and makes more healthy lymphocytes to attack these cells (which are the same type of cells as those affected by CTCL). There is currently a large amount of research into production of new biologic and immune therapies, therefore many emerging therapies of this type are only available through clinical trials.
Chemotherapy is conventionally used to kill cancer cells that are rapidly dividing. This type of therapy has a high rate of side effects because the agents used almost always affect normal dividing cells as well as cancerous ones. This means that patients on chemotherapy must be carefully monitored to avoid too much destruction of healthy cells. For these reasons, chemotherapy is typically reserved for patients in later stages of CTCL to avoid exposure of mostly healthy individuals to the possibility of detrimental side effects. Since chemotherapy has been used for so long, there are many different forms of this treatment class available. This means that patients will have to decide with their doctor as to what specific regiment of chemotherapy will work best for them, even more so than with other types of treatments.
“Cutaneous T-Cell Lymphoma.” Lymphoma Research Foundation, www.lymphoma.org/aboutlymphoma/nhl/ctcl/.
“Extracorporeal Photopheresis.” Extracorporeal Photopheresis - Educational Resources - Community - Multidisciplinary Cutaneous Lymphoma Group - Stanford University School of Medicine, cutaneouslymphoma.stanford.edu/community/photopheresis.html.
Kim, Youn H. “Cutaneous T-Cell Lymphoma Facts.” Leukemia & Lymphoma Society, Leukemia & Lymphoma Society, www.lls.org/sites/default/files/file_assets/cutaneoustcelllymphoma.pdf.