Cutaneous T-cell lymphoma - Lymphoma Association

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Some slow-growing lymphomas do not become obvious until they are more advancedand the lymph nodes, blood or internal organs are affected. However, with treatment,many people with more advanced disease will also have long periods in remission,when they are free of symptoms of the disease.There are some skin lymphomas that are faster growing and more aggressive intheir behaviour from the outset. These develop more rapidly and they require moreintense treatment, more urgently. In addition, some slow-growing skin lymphomasoccasionally change into a faster-growing type. This is called transformation and itis something the medical team check for when you attend the clinic. The treatmentwill become more intense if symptoms or follow-up tests have shown that this hashappened.The rest of the information sheet will describe the symptoms and treatments of thedifferent types of CTCL, starting with the most common type, mycosis fungoides.Mycosis fungoidesMost skin lymphomas develop from T cells and almost two-thirds of people with aT-cell skin lymphoma will have mycosis fungoides. This is therefore the most commonskin lymphoma overall. This lymphoma is a slow-growing or low-grade lymphomaand for many people it will not greatly affect their quality of life or alter their lifeexpectancy.Mycosis fungoides got its odd name because when it was first described at thebeginning of the 19th century the spots were thought to resemble a mushroom-likefungal disease. This is quite wrong of course because it is not caused by a fungus at all,but the name has stuck. The cause of mycosis fungoides is not known.This lymphoma occurs most commonly in people aged between 40 and 60 and itusually develops and progresses slowly over many years or even decades. Abouttwice as many men are affected as women. Most people with mycosis fungoides(about three-quarters) will have early-stage disease when they are first diagnosed.Most people have the ‘classic’ type (see below) and there are three other, rarer forms(see page 12).Classic mycosis fungoidesClassic mycosis fungoides starts as irregularly shaped, oval or annular (ring-like), dryor scaly patches, which are usually flat (see figures 1 and 2, pages 3 and 4). They canbe either discoloured or pale. They can disappear spontaneously, stay the same orslowly enlarge. These are most common on the trunk or buttocks but they can occuranywhere. They are often mistaken for other, more common skin conditions such aseczema or psoriasis, sometimes for many years. The face is only rarely affected.Sometimes similar but thicker and slightly raised areas of skin develop – plaques (seefigure 3, page 4). These plaques can be itchy and they sometimes ulcerate. The mostcommon places for plaques to appear on are the buttocks, in folds of skin and on theface. If they form in areas where there is hair, the hair can be lost. Mycosis fungoidescommonly never progresses beyond this early patch and plaque stage.Cutaneous T-cell lymphomaLYM0157/CTCL/2013v310/18
In a small proportion of people, raised lumps appear on the skin (tumours). Tumourscan ulcerate and weep and this can be painful (see figure 4, page 4).Figure 5: ErythrodermaIn a very few people the skincan become red, thickenedand sore all over. This is callederythroderma (see figure 5). If thishas happened it is likely that therewill be some lymphoma in thelymph nodes too, but mycosisfungoides does not usually involveother internal organs and is rarelyfound in the bone marrow.How is classic mycosis fungoides treated?Mycosis fungoides can be very effectively treated and well controlled, but it has atendency to come back when treatment is stopped. A few people need no treatmentat all at first but a good skincare regime with regular use of moisturisers will help toprevent dryness and irritation. This is all that will be required for some people withstage I disease.If larger areas of skin are affected or the itch is troublesome, mycosis fungoidescan be treated with treatments applied directly to the skin, such as steroid creams,phototherapy with PUVA (a psoralen tablet plus ultraviolet A light) or narrow-band UVB(ultraviolet B light) or topical chemotherapy drugs such as carmustine (BCNU). If tumourshave developed, these can be treated with radiotherapy.If the lymph nodes are involved, if the plaques and tumours are more extensive or ifthe topical treatments are not effective in early-stage disease, a combination of PUVAphototherapy and a drug called interferon alpha (injected just under the skin) or acombination of PUVA phototherapy and a retinoid drug such as bexarotene (Targretin ® )in tablet form might be given. Alternatively, total skin electron beam therapy (TSEBT)might be considered.If mycosis fungoides doesn’t respond to these treatments or if it is at a more advancedstage when it is diagnosed, the following treatments might be considered:● chemotherapy drugs such as methotrexate, chlorambucil, liposomal doxorubicin(Caelyx ® ) or gemcitabine● newer drugs such as the fusion toxin, denileukin diftitox (Ontak ® ) or the histonedeacetylase (HDAC) inhibitor, vorinostat● a combination of chemotherapy drugs (such ‘CHOP’, which is a combination oftenused for people with other types of lymphoma).Cutaneous T-cell lymphomaLYM0157/CTCL/2013v311/18
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Page 3: What causes skin lymphoma?The cause
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Page 8 and 9: What do the ‘stages’ mean?The s
Page 12 and 13: Erythroderma can be treated by extr
Page 14 and 15: people, the average age being about
Page 16 and 17: AcknowledgementsWe are very gratefu
Page 18: Willemze R, et al. WHO-EORTC classi

Cutaneous T-cell lymphoma - Lymphoma Association

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