MYCOSIS FUNGOIDES

Mycosis Fungoides – General Information

Mycosis Fungoides is a very rare form of T-cell lymphoma of the skin (cutaneus). Usually, the disease is slowly progressive and also chronic. These types of lymphomas are included in the Revised European-American Lymphoma classification as low grade T-cell lymphomas, which should be distinguished from other T-cell lymphomas that involve the skin, such as anaplastic large cell lymphoma (CD30 positive), peripheral T-cell lymphoma (CD30 negative, with no epidermal involvement), adult T-cell leukemia/lymphoma (usually with systemic involvement), or subcutaneous panniculitic T-cell lymphoma.
Immunohistochemistry and molecular genetics have revealed aberrant expression of the usual surface antigens of the T lymphocytes. Most cases of Mycosis Fungoides are neoplastic T helper cells which are CD4 positive. These show gene rearrangements of the T cell receptors, usually of the alpha/beta receptors, but occasionally of the gamma/delta receptors.

Extravasating into the dermis, the cells show an affinity for the epidermis, clustering around Langerhans cells as seen microscopically as Pautrier microabscesses. However, the malignant cells that adhere to the skin retain the ability to exit the skin via afferent lymphatic. They travel to lymph nodes and then through efferent lymphatic back to the blood to join the circulating population of CLA-positive T cells. Then, the disease fundamentally is a systemic disease, even when the disease appears to be in an early stage and clinically limited to the skin.

The cause of Mycosis Fungoides is unknown, and it can occur at any age but it is usually common in the 40-60 year old age group. You cannot catch it or give it to anyone else, and it is not hereditary.

Mycosis Fungoides – Symptoms

Mycosis Fungoides – Treatment

Usually, there is no cure for Mycosis Fungoides, so it depends on how far it has spread by the time is diagnosed and the treatment begins, how long a person survives with the disease.When mycosis is in an early stage, a way to treat the disease is to use steroid creams, chemotherapy applied to the skin, or electron beam radiation. This treatment puts the cancer in remission, which often lasts a long time. If the patient’s disease does not respond to the treatment, or the disease got to a tumor stage, treatments such as recombinant alfa Interferon or chemotherapy may be applied. The selection of the specific treatment is based on the clinical stage of the disease. Also needs to be taken in consideration are the facts of such as assessment in prognostic factors, availability of different treatment approaches, patient’s age, social and medical problems, and cost – benefit ratio. For patients with plaque or patch skin involvement (T1 and T2) the treatment plan is limited to topical a therapeutic measure, that’s why people with extracutaneous disease should receive some form of systemic therapy as part of their treatment regimen. Though, there is no evidence that early aggressive systemic therapy is preferable to conservative therapy in the management of limited disease. Despite there are so many years of experience in the treatment of Mycosis Fungoides, well designed, controlled and prospective clinical studies comparing the efficacy of various therapies are lacking.

Ultraviolet light (PUVA) can also help control the condition of the disease and this involves coming to the office, for the PUVA treatment, two to three times a week for ten to fifteen minutes. Another way to treat the disease is with cortisone ointments. Cortisone (steroid) skin preparations are a large family of drugs all similar to Hydrocortisone. The medicines in this family differ primarily by the strength of each compound. Hydrocortisone is a natural chemical made by the body and is one of the weakest members of this group. In general steroid ointments are stronger than steroid creams because the medicine penetrates better when in an ointment form. Examples of these medications include: Aristocort, clobetasol, Cortisone 5 & 10, Diprolene, Elocon, fluocinolone, Halog, Hydrocortisone, triamcinolone, etc. There are many other examples. The systemic treatment also includes oral retinoid, fusion toxin treatment, monoclonal antibody treatment, and systemic chemotherapy with a variety of single agents.

Combination chemotherapy: This generally is not used because the infectious complications and short response duration outweigh the modest response rates (compared to other non-Hodgkin lymphomas). Increased survival is not demonstrated with the use of combination chemotherapy compared to sequential topical agents. Remember that Mycosis Fungoides affects individuals in different ways and that the information given above is generalised.  If you want to know more about this disease, please ask the consultant dermatologist supervising your care.  No special diet is needed, and there need be no restrictions on your activities.