Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)
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.
Overview
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.
State of the art
- 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.
Anatomy and lymph node drainage
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
- Bone marrow (leukaemia, MDS, MPN, myeloma)
- Lymph node germinal centre (lymphomas)
- Spleen
- Blood (leukaemic phase)Sezary syndrome (erythroderma with blood involvement)
- Lytic bone lesions (myeloma)
- Skin and extranodal sitesMycosis fungoides, early stage (patches and plaques) · Mycosis fungoides, tumour stage and large-cell transformation · Folliculotropic mycosis fungoides · Primary cutaneous CD30-positive lymphoproliferative disorders (related)
- cervical
- axillary
- mediastinal
- para-aortic and mesenteric
- inguinal
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: Marginal zone lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD)
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- 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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Topical steroids, nitrogen mustard or bexarotene gel; narrowband UVB or PUVA phototherapy; local radiotherapy; total skin electron beam therapy for widespread disease.
Extracorporeal photopheresis, interferon, oral bexarotene, low-dose methotrexate; mogamulizumab for blood involvement (MAVORIC); brentuximab vedotin for CD30-positive disease (ALCANZA); vorinostat or romidepsin.
Gemcitabine or liposomal doxorubicin chemotherapy; allogeneic stem cell transplant in fit younger patients.
Subtypes & biomarkers
top- 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)
- 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
How often this target appears
- 1806Alibert describes mycosis fungoides
- 1987Extracorporeal photopheresis approved for cutaneous T-cell lymphoma
- 1999Bexarotene approved
- 2006Vorinostat: first HDAC inhibitor approved for any cancer
- 2017ALCANZA: brentuximab vedotin beats standard therapy in CD30-positive disease
- 2018MAVORIC: mogamulizumab approved
What is in development for Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
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.
and how the field plans to fix it →What is being done about thisSide effects and quality of lifeAvailable now- Allogeneic stem cell transplantationStandard of care
- Cardio-oncologyEstablished
- Exercise & lifestyle oncologyEstablished
- Geriatric assessmentEstablished
- IMRT / IGRT (modern external beam)Standard of care
- Oncology nutrition assessment and medical nutrition therapyEstablished
In trialsIdeas and roadmaps- A cheap old tablet to restore appetite
- A coordinated FLASH radiotherapy evidence programme with shared dose-rate standards
- A dedicated programme for cachexia and treatment toxicity research
- A dietitian in every gastrointestinal and head and neck tumour board
- A funded programme of organ-preservation trials to avoid radical surgery
- A lifelong late-effects registry linked to every treatment for adult survivors
Background: CTCAE toxicity grading (grade 3-4 adverse events), De-escalation, escalation and response-adapted therapy, Immune-related adverse events (irAEs), Quality of life, Toxicity grade. Also on OnCo: Side effects by symptom · Immune-related side effects · Toxicity compare · Survivorship planner.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- BC CancerVancouver, BC, CAvia Brentuximab vedotin
- via Allogeneic stem cell transplantation
- German Hodgkin Study GroupCologne, DEvia Brentuximab vedotin
- Indiana University Melvin and Bren Simon Comprehensive Cancer CenterIndianapolis, IN, USNCI comprehensivevia Allogeneic stem cell transplantation
- Juravinski Cancer Centre / Escarpment Cancer Research InstituteHamilton, ON, CAvia Allogeneic stem cell transplantation
- via Allogeneic stem cell transplantation
- via Gemcitabine
- University of Maryland Marlene and Stewart Greenebaum Comprehensive Cancer CenterBaltimore, MD, USNCI comprehensivevia Allogeneic stem cell transplantation
Questions to ask
topQuestions to ask your oncologist about Cutaneous T-cell lymphoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Stage, CD30 expression, CCR4 expression and blood involvement, Large-cell transformation on biopsy, T-cell receptor clonality in skin and blood), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Mycosis fungoides, early stage, Mycosis fungoides, tumour stage and large-cell transformation, Folliculotropic mycosis fungoides.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Early stage (patches and plaques)
- For my situation (early stage (patches and plaques)), which of the standard options do you recommend and why?Why: Guideline options include: Topical steroids, nitrogen mustard or bexarotene gel; narrowband UVB or PUVA phototherapy; local radiotherapy; total skin electron beam therapy for widespread disease.
- Am I a candidate for Bexarotene, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced skin or blood disease
- For my situation (advanced skin or blood disease), which of the standard options do you recommend and why?Why: Guideline options include: Extracorporeal photopheresis, interferon, oral bexarotene, low-dose methotrexate; mogamulizumab for blood involvement (MAVORIC); brentuximab vedotin for CD30-positive disease (ALCANZA); vorinostat or romidepsin.
- Am I a candidate for Methoxsalen (extracorporeal photopheresis), Bexarotene, Methotrexate or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Refractory or transformed
- For my situation (refractory or transformed), which of the standard options do you recommend and why?Why: Guideline options include: Gemcitabine or liposomal doxorubicin chemotherapy; allogeneic stem cell transplant in fit younger patients.
- Am I a candidate for Gemcitabine, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Diagnosis takes years because early disease mimics eczema”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “No cure without transplant”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
8targets
3drugs
8companies
5Latest papers
topQuery for this cancer: (TITLE:"Cutaneous T-cell lymphoma" OR ABSTRACT:"Cutaneous T-cell lymphoma" OR TITLE:"mycosis fungoides and Sezary syndrome" OR ABSTRACT:"mycosis fungoides and Sezary syndrome" OR TITLE:"CTCL" OR ABSTRACT:"CTCL" OR TITLE:"Mycosis fungoides" OR ABSTRACT:"Mycosis fungoides" OR TITLE:"Sezary syndrome" OR ABSTRACT:"Sezary syndrome") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), not a curated reading list.
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