{"entity":{"id":"sezary-syndrome","kind":"cancer","name":"Sezary syndrome","aka":["Sézary syndrome","Sezary disease","Sézary disease","Leukaemic cutaneous T-cell lymphoma","Sezary syndrome (erythroderma with blood involvement)"],"tldr":"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.","summary":"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).\n\nHow 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.\n\nHow common: no figure for the syndrome alone in the sources read; cutaneous T-cell lymphoma overall is about 6 per million a year (Criscione and Weinstock 2007).\n\nTreatment: extracorporeal photopheresis with or without interferon or bexarotene as first-line systemic therapy; mogamulizumab (MAVORIC) or low-dose methotrexate, pralatrexate, brentuximab vedotin in CD30-positive disease, or histone deacetylase inhibitors later; allogeneic stem cell transplant for fit patients with a response; skin-directed therapy and infection control throughout (EORTC 2023).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/S%C3%A9zary_disease","links":[{"label":"NCI PDQ: mycosis fungoides (including Sezary syndrome) treatment","url":"https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"EORTC consensus recommendations for mycosis fungoides and Sezary syndrome, update 2023 (Eur J Cancer)","url":"https://doi.org/10.1016/j.ejca.2023.113343"},{"label":"EORTC consensus recommendations for mycosis fungoides and Sezary syndrome, update 2017 (Eur J Cancer)","url":"https://doi.org/10.1016/j.ejca.2017.02.027"},{"label":"Criscione and Weinstock 2007, Arch Dermatol: incidence of cutaneous T-cell lymphoma in the United States 1973 to 2002","url":"https://doi.org/10.1001/archderm.143.7.854"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["cutaneous-t-cell-lymphoma","peripheral-t-cell-lymphoma","t-cell-prolymphocytic-leukaemia","primary-cutaneous-marginal-zone-lymphoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["mogamulizumab","methoxsalen-ecp","bexarotene","interferon-alfa","methotrexate","pralatrexate","brentuximab-vedotin","romidepsin","vorinostat","resminostat"],"companies":[],"institutions":[],"pathways":[],"terms":["allogeneic-transplant"],"trials":["mavoric","nct04930653","nct02953301"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"A small fraction of cutaneous T-cell lymphoma, which itself had an age-adjusted incidence of 6.4 per million a year in the United States over 1973 to 2002, higher in men (8.7) than women (4.6) and in black (9.0) than white (6.1) Americans (Criscione and Weinstock 2007). No registry figure for Sezary syndrome alone is in the sources read.","subtypes":["Sezary syndrome arising de novo (the usual form)","Sezary syndrome evolving from erythrodermic mycosis fungoides","Sezary syndrome with large-cell transformation"],"biomarkers":["Sezary cell count of 1,000 per microlitre or more, or CD4:CD8 ratio of 10 or more with loss of CD7 or CD26","Clonal T-cell receptor rearrangement matching in skin and blood","CCR4 expression (mogamulizumab target)","CD30 expression (brentuximab vedotin)"],"standardOfCare":[{"setting":"First-line systemic","approach":"Extracorporeal photopheresis with or without interferon or bexarotene (EORTC 2023).","refs":["methoxsalen-ecp","bexarotene","interferon-alfa","cutaneous-t-cell-lymphoma"]},{"setting":"Later lines","approach":"Mogamulizumab (MAVORIC), methotrexate, pralatrexate, brentuximab vedotin for CD30-positive disease, romidepsin or vorinostat; allogeneic transplant for fit responders.","refs":["mogamulizumab","mavoric","methotrexate","pralatrexate","brentuximab-vedotin","romidepsin","vorinostat","allogeneic-transplant"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"cutaneous-t-cell-lymphoma"},"route":"/cancers/sezary-syndrome/","neighbours":{"cancer":[{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","route":"/cancers/cutaneous-t-cell-lymphoma/"},{"id":"peripheral-t-cell-lymphoma","kind":"cancer","name":"Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)","route":"/cancers/peripheral-t-cell-lymphoma/"},{"id":"primary-cutaneous-marginal-zone-lymphoma","kind":"cancer","name":"Primary cutaneous marginal zone lymphoma","route":"/cancers/primary-cutaneous-marginal-zone-lymphoma/"},{"id":"t-cell-prolymphocytic-leukaemia","kind":"cancer","name":"T-cell prolymphocytic leukaemia","route":"/cancers/t-cell-prolymphocytic-leukaemia/"}],"drug":[{"id":"bexarotene","kind":"drug","name":"Bexarotene","route":"/drugs/bexarotene/"},{"id":"brentuximab-vedotin","kind":"drug","name":"Brentuximab vedotin","route":"/drugs/brentuximab-vedotin/"},{"id":"interferon-alfa","kind":"drug","name":"Interferon alfa-2a/2b","route":"/drugs/interferon-alfa/"},{"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":"pralatrexate","kind":"drug","name":"Pralatrexate","route":"/drugs/pralatrexate/"},{"id":"resminostat","kind":"drug","name":"Resminostat","route":"/drugs/resminostat/"},{"id":"romidepsin","kind":"drug","name":"Romidepsin","route":"/drugs/romidepsin/"},{"id":"vorinostat","kind":"drug","name":"Vorinostat","route":"/drugs/vorinostat/"}],"term":[{"id":"allogeneic-transplant","kind":"term","name":"Allogeneic stem cell transplant (allo-SCT)","route":"/terms/allogeneic-transplant/"}],"trial":[{"id":"nct04930653","kind":"trial","name":"Extracorporeal Photopheresis and Mogamulizumab for the Treatment of Erythrodermic Cutaneous T Cell Lymphoma","route":"/trials/nct04930653/"},{"id":"mavoric","kind":"trial","name":"MAVORIC","route":"/trials/mavoric/"},{"id":"nct02953301","kind":"trial","name":"Resminostat for Maintenance Treatment of Patients With Advanced Stage Mycosis Fungoides (MF) or Sézary Syndrome (SS)","route":"/trials/nct02953301/"}]}}