Sezary syndrome
MONDO:0017844Mondo
Findings
No curated finding names Sezary syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Sezary syndrome (SS) is an aggressive form of cutaneous T-cell lymphoma characterized by a triad of erythroderma, lymphadenopathy and circulating atypical lymphocytes (Sezary cells).
Definition from the Mondo Disease Ontology (MONDO:0017844), read 2026-09-29. CC BY 4.0.
Features
29 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal lymphocyte morphologyHPOHP:0004332
- Very frequent (80% to 99% of cases)
- Abnormal T cell morphologyHPOHP:0002843
- Very frequent (80% to 99% of cases)
- Cutaneous T-cell lymphomaHPOHP:0012192
- Very frequent (80% to 99% of cases)
- Dry skinHPOHP:0000958
- Very frequent (80% to 99% of cases)
- ErythrodermaHPOHP:0001019
- Very frequent (80% to 99% of cases)
- LichenificationHPOHP:0100725
- Very frequent (80% to 99% of cases)
- LymphadenopathyHPOHP:0002716
- Very frequent (80% to 99% of cases)
- LymphomaHPOHP:0002665
- Very frequent (80% to 99% of cases)
- Neoplasm of the skinHPOHP:0008069
- Very frequent (80% to 99% of cases)
- PruritusHPOHP:0000989
- Very frequent (80% to 99% of cases)
- AlopeciaHPOHP:0001596
- Frequent (30% to 79% of cases)
- HepatomegalyHPOHP:0002240
- Frequent (30% to 79% of cases)
Show the remaining 17
- ImmunodeficiencyHPOHP:0002721
- Frequent (30% to 79% of cases)
- Increased CD4:CD8 ratioHPOHP:0033221
- Frequent (30% to 79% of cases)
- Nail dystrophyHPOHP:0008404
- Frequent (30% to 79% of cases)
- Palmoplantar keratodermaHPOHP:0000982
- Frequent (30% to 79% of cases)
- SplenomegalyHPOHP:0001744
- Frequent (30% to 79% of cases)
- Abnormal circulating immunoglobulin concentrationHPOHP:0010701
- Occasional (5% to 29% of cases)
Where it sits
Other names
8 names
Resolves to: Sezary syndrome
- Also called
- CTCL / Sezary syndromecutaneous T-cell lymphoma/Sezary syndromeSC)zary syndromeSezary diseaseSezary lymphomaSézary lymphomaSézary syndromeSezary's disease