Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)
Prepared with OnCo (onco.cc/prep/cutaneous-t-cell-lymphoma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
15 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.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?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (early stage (patches and plaques)), which of the standard options do you recommend and why?
- 6.Am I a candidate for Bexarotene, and what side effects should I expect?
- 7.For my situation (advanced skin or blood disease), which of the standard options do you recommend and why?
- 8.Am I a candidate for Methoxsalen (extracorporeal photopheresis), Bexarotene, Methotrexate or related drugs, and what side effects should I expect?
- 9.For my situation (refractory or transformed), which of the standard options do you recommend and why?
- 10.Am I a candidate for Gemcitabine, and what side effects should I expect?
- 11.Are there clinical trials I could join, for example of Soligenix, Mogamulizumab?
- 12.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 13.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 14.I read that “Diagnosis takes years because early disease mimics eczema”. How does that affect my plan?
- 15.I read that “No cure without transplant”. How does that affect my plan?
The words I may hear
- Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields: Early mycosis fungoides is treated on the skin, not through the bloodstream.
- What it costs to aim at a lineage antigen: Almost every lymphoma drug that finds the cancer by a surface marker finds healthy cells carrying the same marker.
- Living with an indolent lymphoma rather than being cured of one: Slow-growing lymphomas are usually controlled rather than cured: treatment works, the disease goes away for a while, and at some point it comes back and is treated again.
- Going back to work after lymphoma, and the money in the meantime: Some people work through lymphoma treatment and some cannot, and the difference is mostly the job rather than the person.
- Fatigue after lymphoma treatment, and why it is a symptom to report: The tiredness that follows lymphoma treatment is not ordinary tiredness and does not reliably improve with rest.
- What the NHS in England funds for lymphoma, appraisal by appraisal: A drug licensed for lymphoma is not automatically available on the NHS: NICE appraises each use separately and can recommend it, fund it for a while through the Cancer Drugs Fund, or refuse it.
- B-cell, T-cell and NK-cell lymphoma: The first thing a lymphoma report says is which kind of lymphocyte the cancer came from.
- A clinical trial or standard treatment in lymphoma: Lymphoma has more trials open to it than almost any other cancer, and in several situations a trial is a reasonable choice beside standard treatment rather than a last resort.
- Indolent and aggressive lymphoma: Lymphomas are split by how fast they grow, and the split decides what happens next.
- Nodal and extranodal lymphoma: A lymphoma that starts in a lymph node is called nodal; one that starts in an organ is called extranodal.
Tests and results to bring
Biomarker results to ask for: 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, Staging that shows no lymphoma outside the skin, which is what makes a cutaneous lymphoma primary, The clinical history and dated photographs, which WHO-HAEM5 calls indispensable because the appearances overlap, CD30, which separates the two CD30-positive lymphoproliferative disorders and decides whether brentuximab vedotin is an option, CD4 against CD8, and alpha-beta against gamma-delta T-cell receptor, which separate the indolent entities from the aggressive ones, A clonal T-cell receptor rearrangement in the skin and, where relevant, in the blood, Blood involvement, which moves the disease from a skin problem to a systemic one.
Scans and tests linked to this cancer: Histopathology & immunohistochemistry, Multidisciplinary tumour boards, Multiparameter flow cytometry MRD, Confocal microscopy and optical coherence tomography for skin (VivaScope, VivoSight, deepLive).
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Early stage (patches and plaques): Topical steroids, nitrogen mustard or bexarotene gel; narrowband UVB or PUVA phototherapy; local radiotherapy; total skin electron beam therapy for widespread disease. (Total skin electron beam therapy, Superficial and orthovoltage radiotherapy for skin cancer, Bexarotene)
- Advanced skin or blood disease: Extracorporeal photopheresis, interferon, oral bexarotene, low-dose methotrexate; mogamulizumab for blood involvement (MAVORIC); brentuximab vedotin for CD30-positive disease (ALCANZA); vorinostat or romidepsin. (Methoxsalen (extracorporeal photopheresis), Bexarotene, Methotrexate, Mogamulizumab, Brentuximab vedotin, Vorinostat, Romidepsin)
- Refractory or transformed: Gemcitabine or liposomal doxorubicin chemotherapy; allogeneic stem cell transplant in fit younger patients. (Gemcitabine, Allogeneic stem cell transplantation)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.