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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Cutaneous T-cell lymphoma is a family of nine lymphomas that start in the skin and mostly stay there, of which mycosis fungoides is by far the commonest. Most of them are long-term skin conditions managed over decades rather than cancers that are cured or not cured, and two of the nine are genuinely aggressive.
What the family is. Primary cutaneous T-cell lymphomas are lymphomas that start in the skin and, for most of their course, stay there. WHO-HAEM5 gives them a family of their own inside the chapter on mature T-cell and NK-cell neoplasms, and lists nine entities in it. The word primary is load-bearing: a systemic lymphoma that has spread to the skin is not a cutaneous lymphoma, is staged differently and is treated differently, so the first job after the biopsy is to show that there is no disease anywhere else.
The nine entities. Mycosis fungoides, which is the commonest by a wide margin and has its own page. The two primary cutaneous CD30-positive lymphoproliferative disorders, lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, which are two ends of one spectrum and both have pages. Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder, which usually presents as a single nodule on the head or neck and behaves benignly. Primary cutaneous acral CD8-positive lymphoproliferative disorder, which WHO-HAEM5 renamed from lymphoma to lymphoproliferative disorder because of how it behaves. Subcutaneous panniculitis-like T-cell lymphoma, which grows in the fat under the skin and can be mistaken for an inflammatory panniculitis. Primary cutaneous gamma/delta T-cell lymphoma and primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, the two genuinely aggressive members. And primary cutaneous peripheral T-cell lymphoma not otherwise specified, a name coined in 2022 for the rare cases that fit none of the others.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |
| 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. | not mapped |
| Refractory or transformed | Gemcitabine or liposomal doxorubicin chemotherapy; allogeneic stem cell transplant in fit younger patients. | not mapped |