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ORPHA:3162Disease
Also called Sézary lymphoma
What it is
Sézary syndrome (SS) is an aggressive form of cutaneous T-cell lymphoma characterized by a triad of erythroderma, lymphadenopathy and circulating atypical lymphocytes (Sézary cells).
Key facts
- Prevalence
- <1 / 1 000 000 (annual incidence, United States)
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
10Common30–79%
7Sometimes5–29%
12- Abnormal immunoglobulin level
- Abnormality of the pleura
- Chills
- Ectropion
- Edema
- Gangrene
- Hypothermia
- Irregular hyperpigmentation
and 4 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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