September 27, 2014 | by Kim Proescholdt
Cutaneous T cell lymphomas are types of non-Hodgkin lymphoma that arise when infection-fighting white blood cells in the lymphatic system – called lymphocytes – become malignant and affect the skin. The result is rashes and, sometimes, tumors, which can be mistaken for other dermatological conditions. In a small number of people, the disease may progress to the lymph nodes or internal organs, causing serious complications.
Here Jasmine Zain, M.D., associate clinical professor and director of City of Hope’s T Cell Lymphoma Program, discusses how in recent years, greater research efforts, advanced treatment options and more collaboration among physicians have contributed to better care and outcomes for patients, and helped many to return to a normal life.
What is cutaneous T cell lymphoma (CTCL) and what are the symptoms?
CTCL is a rare form of lymphoma that arises primarily in the skin. It is not to be confused with the more common forms of skin cancer that include melanoma and squamous cell carcinoma. Lymphomas are cancers of the lymphoid system and usually arise in lymph nodes. However, with skin being the largest lymphoid organ in the body and our first line of defense against the outside environment, occasionally it becomes the site of lymphoma formation.
Unlike nodal lymphomas, skin lymphomas tend to be of the T cell type, though there are some forms of B cell lymphomas (cutaneous B cell lymphomas, or CBCL) that can arise in the skin, as well. There are several types of cutaneous T and B cell lymphomas. They are approached, staged and treated in unique ways as compared to other types of lymphomas.
Mycosis fungoides is the most common type of CTCL. A primary symptom is a rash that arises initially in areas of the skin that are not normally exposed to sunlight. They look like eczematous rashes and can be difficult to distinguish from benign skin conditions like eczema or psoriasis, even with a biopsy. This type of lymphoma remains confined to the skin and does not involve internal organs until late stages of the disease. The clinical course is indolent with most of the patients surviving 20 years or more.
Are there any known risk factors for CTCL?
There are no known risk factors for CTCL. In particular, there is no association with exposure to sunlight or UV radiation. It cannot be transmitted to anyone by contact or any other means.
How is CTCL treated at City of Hope? Are there any new advances?
City of Hope is in the unique position to offer the whole spectrum of therapeutic options for CTCL including light therapy, photophreisis, chemotherapy and stem cell transplantation. There is a large portfolio of clinical trials aimed at innovative and targeted therapies for CTCL based upon basic science research being done by our scientists. Treatment of CTCL requires the expertise of many specialists including dermatology, oncology, radiation oncology and dermatopathology. At City of Hope, we employ a multidisciplinary approach for the treatment of these patients where all the specialists work together to establish the best treatment plan possible in a timely manner. This approach has been well established in some of the other large centers for CTCL in the country.
What’s new in CTCL research?
The last few years have seen some exciting new advances in the treatment of CTCL. These include the Federal Drug Administration’s approval of two epignetic agents for CTCL – vorinostat and romidepsin. These agents modify genetic expression of abnormal cancerous genes and have shown remarkable and sustained activity in the treatment of CTCL. In addition, several newer agents that have been approved for the treatment of peripheral T cell lymphomas have also shown excellent activity in CTCL and are in clinical use. These include pralatrexate and brentuximab vedotin. City of Hope was involved in the conduct of the pivotal trials for all the approval of these agents, and physician scientists here continue to explore molecular pathways that can be targeted for the treatment of CTCL.
Why did you choose this field? What inspires you to do the work you do?
I got involved in the care of CTCL patients early on in my career and have been doing so now for over 15 years. I started working with CTCL because my division chief asked me to do so, and it was the best decision of my life. It has become the most exciting part of my academic career.
My biggest motivation remains my patients. CTCL patients have the unique challenge of having their disease on the “outside” of their body, affecting their appearance, their life and even their relationships. Since these are indolent disease, the patients have this disease for a long time, sometimes even the rest of their lives. And they have to continue to function in spite of it. I have seen examples of incredible strength of humanity and patience in these patients. Some of the most rewarding transplant outcomes for me have been with CTCL patients who have gone on to live normal lives.
What advice do you have for a patient recently diagnosed with CTCL?
These are rare diseases and most physicians see very few cases in their lifetime. The best advice is to get a second opinion at a center where the dermatologists, oncologists and pathologists have had experience in CTCL. A review of the biopsy and meeting with an expert will help you understand your disease and treatment options better. Most experts are willing to work with your local doctor to provide the best care possible and to ensure that you don’t miss out on any innovative therapies. The Cutaneous Lymphoma Foundation is also an excellent resource for patients looking for information on CTCL and the experts that treat the disease in their area.
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Learn more about non-Hodgkin lymphoma research and treatment at City of Hope.