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6 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.
Sezary syndrome is the leukaemic form of skin lymphoma: the whole skin turns red and scaly, the lymph nodes swell, and malignant T cells circulate in the blood. It is treated to control rather than cure, with photopheresis, the antibody mogamulizumab, and drugs such as bexarotene and interferon, and a stem cell transplant is the only treatment that can cure it in fit patients.
WHO-HAEM5 keeps Sezary syndrome as a distinct entity from mycosis fungoides, defined by the triad of erythroderma, generalised lymphadenopathy and clonal neoplastic T cells in skin, nodes and blood, with a blood tumour burden of 1,000 or more Sezary cells per microlitre or equivalent flow cytometry criteria (Alaggio 2022; EORTC 2023). The EORTC consensus recommendations, updated in 2017 and 2023, set the stage-adapted treatment for mycosis fungoides and Sezary syndrome, noting that controlled studies remain few; the 2023 update incorporates chlormethine, brentuximab vedotin and mogamulizumab, recommends pegylated interferon after the withdrawal of unpegylated interferons, and adds guidance on supportive care and older patients (EORTC 2017; EORTC 2023). Mogamulizumab, the anti-CCR4 antibody, is the drug with a randomised trial in this setting (MAVORIC, linked here).
How it differs from its parent: the parent page covers mycosis fungoides, in which most patients have a normal life expectancy with skin-directed treatment; Sezary syndrome is advanced-stage disease by definition, blood-borne, immunosuppressing and life-shortening, treated systemically from the outset.
| Setting | Approach | Guideline |
|---|---|---|
| First-line systemic | Extracorporeal photopheresis with or without interferon or bexarotene (EORTC 2023). | not mapped |
| Later lines | Mogamulizumab (MAVORIC), methotrexate, pralatrexate, brentuximab vedotin for CD30-positive disease, romidepsin or vorinostat; allogeneic transplant for fit responders. | not mapped |
| Sezary syndrome: treatment aimed at the blood as well as the skin | Sezary syndrome is the leukaemic form: erythroderma covering most of the body, lymphadenopathy, intractable itch and a clone of malignant T cells in the blood. It is treated as advanced disease from the start, and treatment has to reduce the blood compartment, not only the skin. Extracorporeal photopheresis is the treatment most specific to it: the patient's white cells are drawn off, exposed to methoxsalen and ultraviolet A light, and returned, usually on two consecutive days every two to four weeks. It is well tolerated, works slowly over months, and is often combined with interferon alfa or bexarotene. It has been approved for the skin manifestations of cutaneous T-cell lymphoma in the United States since 1999. Mogamulizumab is the systemic drug of choice where the blood is heavily involved, because in MAVORIC the response rate in the blood compartment was 68 per cent, far above its skin response; median progression-free survival was 7.7 against 3.1 months for vorinostat. Other options are bexarotene, interferon, low-dose methotrexate, romidepsin, alemtuzumab at low subcutaneous dose, chlorambucil with prednisolone for an older patient, and allogeneic transplant with reduced-intensity conditioning for fit younger patients, which is the only treatment that produces durable remission. Skin care, control of itch and prevention of staphylococcal sepsis matter at least as much as the lymphoma treatment; erythrodermic skin loses heat, fluid and protein and is an open door to infection. | not mapped |