# Sezary syndrome

Source: https://onco.cc/cancers/sezary-syndrome/  
OnCo record `sezary-syndrome` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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.

How 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).

Treatment: 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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Sézary syndrome; Sezary disease; Sézary disease; Leukaemic cutaneous T-cell lymphoma; Sezary syndrome (erythroderma with blood involvement)
- Tags: subtype-page; wave4; haematologic; rare
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/sezary-syndrome/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/sezary-syndrome/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/sezary-syndrome/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/sezary-syndrome/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/sezary-syndrome/#treating-it [2 settings, 2 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/sezary-syndrome/#evidence [3 trials]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/sezary-syndrome/#science [3 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/sezary-syndrome/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/sezary-syndrome/#living-with-it [11 questions, 5 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/sezary-syndrome/coming/ [10 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/sezary-syndrome/data/ [32 connected records]

## Standard of care

- First-line systemic: Extracorporeal photopheresis with or without interferon or bexarotene (EORTC 2023). ([Methoxsalen (extracorporeal photopheresis)](https://onco.cc/drugs/methoxsalen-ecp/), [Bexarotene](https://onco.cc/drugs/bexarotene/), [Interferon alfa-2a/2b](https://onco.cc/drugs/interferon-alfa/), [Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)](https://onco.cc/cancers/cutaneous-t-cell-lymphoma/))
- Later lines: Mogamulizumab (MAVORIC), methotrexate, pralatrexate, brentuximab vedotin for CD30-positive disease, romidepsin or vorinostat; allogeneic transplant for fit responders. ([Mogamulizumab](https://onco.cc/drugs/mogamulizumab/), [MAVORIC](https://onco.cc/trials/mavoric/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [Pralatrexate](https://onco.cc/drugs/pralatrexate/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Romidepsin](https://onco.cc/drugs/romidepsin/), [Vorinostat](https://onco.cc/drugs/vorinostat/), [Allogeneic stem cell transplant (allo-SCT)](https://onco.cc/terms/allogeneic-transplant/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/S%C3%A9zary_disease
- NCI PDQ: mycosis fungoides (including Sezary syndrome) treatment: https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- EORTC consensus recommendations for mycosis fungoides and Sezary syndrome, update 2023 (Eur J Cancer): https://doi.org/10.1016/j.ejca.2023.113343
- EORTC consensus recommendations for mycosis fungoides and Sezary syndrome, update 2017 (Eur J Cancer): https://doi.org/10.1016/j.ejca.2017.02.027
- Criscione and Weinstock 2007, Arch Dermatol: incidence of cutaneous T-cell lymphoma in the United States 1973 to 2002: https://doi.org/10.1001/archderm.143.7.854

## Connected records

- cancers: [Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)](https://onco.cc/cancers/cutaneous-t-cell-lymphoma/), [Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)](https://onco.cc/cancers/peripheral-t-cell-lymphoma/), [Primary cutaneous marginal zone lymphoma](https://onco.cc/cancers/primary-cutaneous-marginal-zone-lymphoma/), [T-cell prolymphocytic leukaemia](https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/)
- drugs: [Bexarotene](https://onco.cc/drugs/bexarotene/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Interferon alfa-2a/2b](https://onco.cc/drugs/interferon-alfa/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [Methoxsalen (extracorporeal photopheresis)](https://onco.cc/drugs/methoxsalen-ecp/), [Mogamulizumab](https://onco.cc/drugs/mogamulizumab/), [Pralatrexate](https://onco.cc/drugs/pralatrexate/), [Resminostat](https://onco.cc/drugs/resminostat/), [Romidepsin](https://onco.cc/drugs/romidepsin/), [Vorinostat](https://onco.cc/drugs/vorinostat/)
- terms: [Allogeneic stem cell transplant (allo-SCT)](https://onco.cc/terms/allogeneic-transplant/)
- trials: [Extracorporeal Photopheresis and Mogamulizumab for the Treatment of Erythrodermic Cutaneous T Cell Lymphoma](https://onco.cc/trials/nct04930653/), [MAVORIC](https://onco.cc/trials/mavoric/), [Resminostat for Maintenance Treatment of Patients With Advanced Stage Mycosis Fungoides (MF) or Sézary Syndrome (SS)](https://onco.cc/trials/nct02953301/)

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JSON: https://onco.cc/api/v1/entities/sezary-syndrome.json