Mycosis fungoides, the most common type of cutaneous T cell lymphoma (CTCL), is a slow growing form of cancer in which some of the body's white blood cells become malignant. These abnormal cells are drawn to the skin and some are deposited there. They are a special type of white blood cell called T-lymphocytes (T-Cells). T-cells regulate the body's immune system in its job of fighting infections and other harmful things in the body.
Cutaneous T cell lymphoma usually develops slowly over many years. In the early stages skin may develop dry, dark patches on the skin, sometimes itchy, sometimes not. Often, it is misdiagnosed as psoriasis or eczema at first and only recognized after several biopsies.
Cancers of the lymphatic system are called "lymphomas". The most well known lymphoma is Hodgkin's disease. All the other lymphomas are called non-Hodgkin's lymphoma. CTCL is a sub-type of non-Hodgkin's lymphoma. Unlike the vast majority of lymphomas, CTCL is made from T-Cells .
CTCL isn't one disease but a collection of related cancers of the lymphatic system. The most common type of CTCL is still sometimes known by its old name "mycosis fungoides". This name referred to mushroom fungus look of the skin only seen in advanced disease. About 10% of CTCL patients instead have widespread redness of the skin and abnormal blood counts (the Sezary Syndrome). Some cases of CTCL may develop from white spots ("hypopigmented MF") or red sores (lymphomatoid papulosis). There are other, even rarer ways for CTCL to appear.
If the disease progresses unchecked, raised growths may form on the skin after a period of years. If they become tumors, the risk increases that tumors will form in the lymph glands or other organs in the body. Even in very early CTCL these abnormal T-Cells are probably present in tiny amounts through-out the bloodstream from the beginning, but when present in large amounts it indicated more advanced disease.
Most cancers are treated with surgery and chemotherapy. Unfortunately, slow growing forms of cancer, such as CTCL, are less curable with chemotherapy than fast growing ones, and surgery is usually not an option. So there isn't one "right" treatment for all patients with CTCL. Treatment is based mostly on the size, extent and places the CTCL is found and the patient's age and overall health.
For early and very limited CTCL treatment with a potent topical "steroid cream" (Diprolene, Temovate, Lidex, and many other brands) is often the initial choice. Phototherapy (PUVA, Narrow band or broadband UVB) is useful if larger body areas are involved. Topical chemotherapy with agents such as nitrogen mustard (Mustergen) or BCNU (Carmustine) is more aggressive and less widely used. Targretin gel is a recent addition and can be useful.
For treatment failures or more advanced CTCL a systemic therapy is used. Choices include Bexarotene capsules (Targretin), intravenous fusion protein (Ontak), Interferon-alpha or methotrexate (Rheumatrex). Sometimes these systemic treatments are combined together or with external treatments to produce better results. More advanced disease may also be treated with total skin electron beam radiation therapy, but this is often saved as it can not be repeated. Sezary syndrome, and some cases of CTCL, is treated with extracorporeal photochemotherapy. Further research is ongoing to determine the optimal combinations for the different stages of CTCL. For severe disease that has failed other treatments bone marrow transplantation has been used.
Early disease is usually treated best by your dermatologist. Advanced disease should be treated in a center that specializes in this condition. IT IS IMPORTANT YOU DO NOT ACCEPT THE DIRE PROGNOSIS given in older medical literature you may find in a public library. Patients diagnosed at early stages can usually expect to live a normal life span and die of unrelated causes. Treatment is effective, but may need to be continued for quite a while. For some, relapses occur, and treatment must either be lifelong in order to control the disease, or it must be repeated each time a relapse occurs.
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