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ORPHA:3162

Sézary syndrome

low confidenceDisorder

Also known as: Sézary lymphoma

Publications

6,286

Trials

142

Interventional, condition-specific

Researchers

1,293

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

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).

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (7)

CTCL / Sezary syndrome · SC)zary syndrome · Sezary disease · Sezary lymphoma · Sezary syndrome · Sezary's disease · cutaneous T-cell lymphoma/Sezary syndrome

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    6,286 matched papers (2,987 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    142 matched on ClinicalTrials.gov (8 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

6,286

6,286 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

6,286 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

2,987 in the last 10 years · low confidence

Phrase hits: 6,286 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,293

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Bagot M13 papers · 2026

    Department of Dermatology, Saint-Louis Hospital, AP-HP, Paris, France.

    Papers in Europe PMC
  2. 02
    Guenova E13 papers · 2026

    University Institute and Clinic for Immuno-dermatology, Medical Faculty, Johannes Kepler University, Secretary of the EORTC Cutaneous Lymphoma Tumor Group, Linz, Austria.

    Papers in Europe PMC
  3. 03
    Nicolay JP11 papers · 2026

    Department of Dermatology, Venereology and Allergology, University Medical Center Mannheim/University of Heidelberg, Mannheim, Germany.

    Papers in Europe PMC
  4. 04
    Quaglino P10 papers · 2026

    Dermatologic Clinic, Department of Medical Sciences, University of Turin Medical School, Torino, Italy.

    Papers in Europe PMC
  5. 05
    Stadler R10 papers · 2026

    Department of Dermatology, Johannes Wesling Medical Center, University Clinic of the Ruhr University of Bochum.

    Papers in Europe PMC
  6. 06
    de Masson A9 papers · 2026

    Department of Dermatology, Saint-Louis Hospital, AP-HP, National Reference Center for Cutaneous Lymphomas, Institut du Cancer AP-HP Nord, Université Paris Cité, Paris, France.

    Papers in Europe PMC
  7. 07
    Foss F9 papers · 2026

    Section of Hematology, Department of Internal Medicine, Yale School of Medicine, New Haven, CT, USA.

    Papers in Europe PMC
  8. 08
    Assaf C8 papers · 2026

    Department of Dermatology and Venereology, HELIOS Klinikum Krefeld and Institute for Molecular Medicine, Medical School Hamburg, Hamburg, Germany.

    Papers in Europe PMC
  9. 09
    Papadavid E8 papers · 2026

    Department of Dermatology, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece.

    Papers in Europe PMC
  10. 10
    Beylot-Barry M7 papers · 2026

    Department of Dermatology, CHU de Bordeaux, BoRdeaux Institute of Oncology, BRIC INSERM U1312, INSERM BoRdeaux Institute of Oncology, Team 5, Université de Bordeaux, Bordeaux, France.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

142

interventional trials for this specific condition

142 interventional trials matched this specific condition name; 8 currently recruiting in our sample.

Data as of 27 July 2026

142 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 98.9th percentile).

low confidence · 98.9th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

142 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

18 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Sézary syndrome" OR "Sézary lymphoma" OR "CTCL / Sezary syndrome" OR "SC)zary syndrome" OR "Sezary disease" OR "Sezary lymphoma" OR "Sezary syndrome" OR "Sezary's disease" OR "cutaneous T-cell lymphoma/Sezary syndrome"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Sézary syndrome" OR "Sézary lymphoma" OR "CTCL / Sezary syndrome" OR "SC)zary syndrome" OR "Sezary disease" OR "Sezary lymphoma" OR "Sezary syndrome" OR "Sezary's disease" OR "cutaneous T-cell lymphoma/Sezary syndrome"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 142 interventional · 18 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is short or not clearly distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (6286) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-26T22:21:01.421Z