{"entity":{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","aka":["CTCL","Mycosis fungoides","Sezary syndrome"],"tldr":"Mycosis fungoides is a lymphoma that lives in the skin, looking like eczema or psoriasis for years before it is diagnosed. It is treated with creams, light and skin-directed radiotherapy for as long as possible, then with antibodies such as mogamulizumab and brentuximab vedotin when it spreads to the blood or lymph nodes.","summary":"Mycosis fungoides is an epidermotropic T-cell lymphoma that progresses from patches to plaques to tumours over many years; Sezary syndrome is its leukaemic form with erythroderma and circulating malignant T cells. Staging by skin, node, blood and viscera drives treatment. Early-stage disease is treated with topical steroids, nitrogen mustard, bexarotene gel, phototherapy (narrowband UVB or PUVA), local radiotherapy and total skin electron beam therapy, which controls the whole skin surface. Advanced disease receives extracorporeal photopheresis, interferon, oral bexarotene, methotrexate, the HDAC inhibitors vorinostat and romidepsin, brentuximab vedotin for CD30-positive disease (ALCANZA) and mogamulizumab, an anti-CCR4 antibody that is especially effective in Sezary syndrome (MAVORIC); allogeneic transplant is the only curative option for young patients with advanced disease.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Mycosis_fungoides","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Mycosis_fungoides"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"haematologic","burden":"The commonest lymphoma of the skin, about one new case per 100,000 people a year; most patients have patches and plaques for decades and die of something else, while the minority with tumours, blood involvement or large-cell transformation have a median survival of a few years.","subtypes":["Mycosis fungoides, early stage (patches and plaques)","Mycosis fungoides, tumour stage and large-cell transformation","Folliculotropic mycosis fungoides","Sezary syndrome (erythroderma with blood involvement)","Primary cutaneous CD30-positive lymphoproliferative disorders (related)"],"biomarkers":["Stage (skin, node, blood, viscera)","CD30 expression (brentuximab vedotin)","CCR4 expression and blood involvement (mogamulizumab)","Large-cell transformation on biopsy","T-cell receptor clonality in skin and blood"],"standardOfCare":[{"setting":"Early stage (patches and plaques)","approach":"Topical steroids, nitrogen mustard or bexarotene gel; narrowband UVB or PUVA phototherapy; local radiotherapy; total skin electron beam therapy for widespread disease.","refs":["total-skin-electron-therapy","superficial-radiotherapy","bexarotene"]},{"setting":"Advanced skin or blood disease","approach":"Extracorporeal photopheresis, interferon, oral bexarotene, low-dose methotrexate; mogamulizumab for blood involvement (MAVORIC); brentuximab vedotin for CD30-positive disease (ALCANZA); vorinostat or romidepsin.","refs":["methoxsalen-ecp","bexarotene","methotrexate","mogamulizumab","brentuximab-vedotin","vorinostat","romidepsin"]},{"setting":"Refractory or transformed","approach":"Gemcitabine or liposomal doxorubicin chemotherapy; allogeneic stem cell transplant in fit younger patients.","refs":["gemcitabine","allogeneic-hsct"]}],"stateOfArt":["Mogamulizumab and brentuximab vedotin were the first targeted antibodies to beat standard care in randomised trials of this disease.","Skin-directed therapy, including total skin electron beam, keeps most patients well for years without systemic drugs.","Hypericin ointment activated by visible light (HyBryte) completed phase 3 and is under review."],"history":[{"year":1806,"title":"Alibert describes mycosis fungoides","refs":[]},{"year":1987,"title":"Extracorporeal photopheresis approved for cutaneous T-cell lymphoma","refs":["methoxsalen-ecp"]},{"year":1999,"title":"Bexarotene approved","refs":["bexarotene"]},{"year":2006,"title":"Vorinostat: first HDAC inhibitor approved for any cancer","refs":["vorinostat"]},{"year":2017,"title":"ALCANZA: brentuximab vedotin beats standard therapy in CD30-positive disease","refs":["brentuximab-vedotin"]},{"year":2018,"title":"MAVORIC: mogamulizumab approved","refs":["mogamulizumab"]}],"pipeline":["soligenix","mogamulizumab"],"openProblems":["Diagnosis takes years because early disease mimics eczema.","No cure without transplant.","Quality of life with itch and visible disease is under-measured in trials."],"parent":"peripheral-t-cell-lymphoma"},"route":"/cancers/cutaneous-t-cell-lymphoma/","neighbours":{"drug":[{"id":"bexarotene","kind":"drug","name":"Bexarotene","route":"/drugs/bexarotene/"},{"id":"brentuximab-vedotin","kind":"drug","name":"Brentuximab vedotin","route":"/drugs/brentuximab-vedotin/"},{"id":"gemcitabine","kind":"drug","name":"Gemcitabine","route":"/drugs/gemcitabine/"},{"id":"methotrexate","kind":"drug","name":"Methotrexate","route":"/drugs/methotrexate/"},{"id":"methoxsalen-ecp","kind":"drug","name":"Methoxsalen (extracorporeal photopheresis)","route":"/drugs/methoxsalen-ecp/"},{"id":"mogamulizumab","kind":"drug","name":"Mogamulizumab","route":"/drugs/mogamulizumab/"},{"id":"romidepsin","kind":"drug","name":"Romidepsin","route":"/drugs/romidepsin/"},{"id":"vorinostat","kind":"drug","name":"Vorinostat","route":"/drugs/vorinostat/"}],"company":[{"id":"soligenix","kind":"company","name":"Soligenix","route":"/companies/soligenix/"}],"technology":[{"id":"allogeneic-hsct","kind":"technology","name":"Allogeneic stem cell transplantation","route":"/technologies/allogeneic-hsct/"},{"id":"superficial-radiotherapy","kind":"technology","name":"Superficial and orthovoltage radiotherapy for skin cancer","route":"/technologies/superficial-radiotherapy/"},{"id":"total-skin-electron-therapy","kind":"technology","name":"Total skin electron beam therapy","route":"/technologies/total-skin-electron-therapy/"}],"cancer":[{"id":"peripheral-t-cell-lymphoma","kind":"cancer","name":"Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)","route":"/cancers/peripheral-t-cell-lymphoma/"}]}}