Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (765 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
A form of cutaneous T-cell lymphoma manifested by generalized exfoliative erythroderma; pruritus; peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear (cerebriform) cells in the skin, lymph nodes, and peripheral blood (Sezary cells).
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Syndrome, Sezary; Erythroderma, Sezary; Sezary Erythroderma; Sezary's Lymphoma; Lymphoma, Sezary's; Sezary Lymphoma; Sezarys Lymphoma.
Where it fits in the MeSH classification
- Broader topics
- Lymphoma, T-Cell, Cutaneous; Lymphoproliferative Disorders
Related concepts
- Erythroderma
- Pruritus
- Lymph Nodes
Research landscape
PubMed indexes 2,144 records tagged with the MeSH heading “Sezary Syndrome” (counts retrieved 2026-10-29). 404 of them (18.8%) were published from 2020 onward, and the busiest period so far is through 1999 with 837 records.
Reviews account for 374 records (17.4%) and meta-analyses for 5. Randomized controlled trials number 11 (0.5%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 837 |
| 2000-2009 | 391 |
| 2010-2019 | 512 |
| 2020 onward | 404 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 374 | 17.4% |
| Meta-analyses | 5 | 0.2% |
| Randomized controlled trials | 11 | 0.5% |
Method: PubMed query "Sezary Syndrome"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in cancer and oncology
Cancer research spans very different kinds of evidence. Laboratory studies in cell lines and animal models reveal mechanisms but often do not translate to patients. Early-phase trials mainly test safety and dosing, while phase 3 randomized trials and their meta-analyses are the usual basis for treatment conclusions. Outcomes also differ in meaning: tumour shrinkage, progression-free survival and overall survival are not interchangeable, and screening studies can be distorted by lead-time and overdiagnosis. When reading a result, check the cancer type and stage studied, the comparison treatment, and which outcome was measured.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| High-resolution analysis of the human T-cell receptor repertoire | 2015 | Research article Design not recorded in structured metadata | 100 | cc by PMID 26324409 · DOI · PMC4569693 |
| Genetic rearrangements result in altered gene expression and novel fusion transcripts in Sézary syndrome | 2017 | Research article Design not recorded in structured metadata | 40 | cc by PMID 28489605 · DOI · PMC5503638 |
| Blood and skin-derived Sezary cells: differences in proliferation-index, activation of PI3K/AKT/mTORC1 pathway and its prognostic relevance | 2019 | Research article Design not recorded in structured metadata | 38 | cc by PMID 30518812 · DOI · PMC6756225 |
| Mapping malignant T-cell states and immune circuits in Sézary syndrome by single-cell analysis | 2026 | Research article Design not recorded in structured metadata | 3 | cc by PMID 41773808 · DOI · PMC13505662 |
High-resolution analysis of the human T-cell receptor repertoire [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Unbiased dissection of T-cell receptor (TCR) repertoire diversity at the nucleotide level could provide important insights into human immunity.
- Closing excerpt
- TCR-LA-MC PCR allows ultra-deep data access to both physiological TCR diversity and mechanisms influencing clonality in all clinical settings with restricted or distorted TCR repertoires.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (94 words)
Unbiased dissection of T-cell receptor (TCR) repertoire diversity at the nucleotide level could provide important insights into human immunity. Here we show that TCR ligation-anchored-magnetically captured PCR (TCR-LA-MC PCR) identifies TCR α- and β-chain diversity without sequence-associated or quantitative restrictions in healthy and diseased conditions. TCR-LA-MC PCR identifies convergent recombination events, classifies different stages of cutaneous T-cell lymphoma in vivo and demonstrates TCR reactivation after in vitro cytomegalovirus stimulation. TCR-LA-MC PCR allows ultra-deep data access to both physiological TCR diversity and mechanisms influencing clonality in all clinical settings with restricted or distorted TCR repertoires.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 26324409 · DOI · PMC4569693
Genetic rearrangements result in altered gene expression and novel fusion transcripts in Sézary syndrome [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Sézary syndrome (SS) is an aggressive, leukemic cutaneous T-cell lymphoma variant.
- Closing excerpt
- Our results not only underscored the genomic complexity of the Sézary cancer cell genome but also showed an unpreceded large variety of novel gene rearrangements resulting in fusions transcripts and ectopically expressed genes.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (186 words)
Sézary syndrome (SS) is an aggressive, leukemic cutaneous T-cell lymphoma variant. Molecular pathogenesis of SS is still unclear despite many studies on genetic alterations, gene expression and epigenetic regulations. Through whole genome and transcriptome next generation sequencing nine Sézary syndrome patients were analyzed in terms of copy number variations and rearrangements affecting gene expression. Recurrent copy number variations were detected within 8q (MYC, TOX), 17p (TP53, NCOR1), 10q (PTEN, FAS), 2p (DNMT3A), 11q (USP28), 9p (CAAP1), but no recurrent rearrangements were identified. However, expression of five genes involved in rearrangements (TMEM244, EHD1, MTMR2, RNF123 and TOX) was altered in all patients. Fifteen rearrangements detected in Sézary syndrome patients and SeAx resulted in an expression of new fusion transcripts, nine of them were in frame (EHD1-CAPN12, TMEM66-BAIAP2, MBD4-PTPRC, PTPRC-CPN2, MYB-MBNL1, TFG-GPR128, MAP4K3-FIGLA, DCP1A-CCL27, MBNL1-KIAA2018) and five resulted in ectopic expression of fragments of genes not expressed in normal T-cells (BAIAP2, CPN2, GPR128, CAPN12, FIGLA). Our results not only underscored the genomic complexity of the Sézary cancer cell genome but also showed an unpreceded large variety of novel gene rearrangements resulting in fusions transcripts and ectopically expressed genes.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 28489605 · DOI · PMC5503638
Blood and skin-derived Sezary cells: differences in proliferation-index, activation of PI3K/AKT/mTORC1 pathway and its prognostic relevance [3]
Abstract reading summary — source excerpts
- Opening excerpt
- Sézary syndrome (SS) is a rare and aggressive variant of Cutaneous T-Cell Lymphoma characterized by neoplastic distribution mainly involving blood, skin, and lymph-node.
- Closing excerpt
- These data shed light on effects in vivo of skin-SS cells interaction underlying the prognostic and therapeutic relevance of mTORC1 pathway in SS.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (200 words)
Sézary syndrome (SS) is a rare and aggressive variant of Cutaneous T-Cell Lymphoma characterized by neoplastic distribution mainly involving blood, skin, and lymph-node. Although a role of the skin microenvironment in SS pathogenesis has long been hypothesized, its function in vivo is poorly characterized. To deepen this aspect, here we compared skin to blood-derived SS cells concurrently obtained from SS patients highlighting a greater proliferation-index and a PI3K/AKT/mTORC1 pathway activation level, particularly of mTOR protein, in skin-derived-SS cells. We proved that SDF-1 and CCL21 chemokines, both overexpressed in SS tissues, induce mTORC1 signaling activation, cell proliferation and Ki67 up-regulation in a SS-derived cell line and primary-SS cells. In a cohort of 43 SS cases, we observed recurrent copy number variations (CNV) of members belonging to this cascade, namely: loss of LKB1 (48%), PTEN (39%) and PDCD4 (35%) and gains of P70S6K (30%). These alterations represent druggable targets unraveling new therapeutic treatments as metformin here evaluated in vitro. Moreover, CNV of PTEN, PDCD4, and P70S6K, evaluated individually or in combination, are associated with reduced survival of SS patients. These data shed light on effects in vivo of skin-SS cells interaction underlying the prognostic and therapeutic relevance of mTORC1 pathway in SS.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 30518812 · DOI · PMC6756225
Mapping malignant T-cell states and immune circuits in Sézary syndrome by single-cell analysis [4]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundSézary syndrome (SS) and leukaemic mycosis fungoides (MF) are aggressive cutaneous T-cell lymphomas (CTCLs) with poor prognosis and limited treatment options.
- Closing excerpt
- These findings lay the groundwork for subtype-specific and microenvironment-informed therapeutic strategies in Sézary syndrome, with potential implications for guiding targeted therapy selection but require validation in larger, independent cohorts.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (285 words)
BackgroundSézary syndrome (SS) and leukaemic mycosis fungoides (MF) are aggressive cutaneous T-cell lymphomas (CTCLs) with poor prognosis and limited treatment options. Single-cell RNA sequencing (scRNA-seq) offers an opportunity to dissect malignant heterogeneity and immune dysregulation yet has been underutilized in leukaemic CTCL.ObjectivesTo define transcriptional heterogeneity within malignant T cells, identify immunomodulatory signals shaping the tumour microenvironment and uncover actionable vulnerabilities in SS and leukaemic MF.MethodsWe analysed 144,321 peripheral blood mononuclear cells from 22 patients (SS or leukaemic MF) and 7 healthy controls using scRNA-seq, with CITE-seq and TCR-seq in subsets. Public and prospectively collected datasets were integrated after rigorous per-sample quality control, batch correction and malignancy assignment. Differential gene expression and cell-cell communication analyses were performed using complementary modelling approaches with correction for sparsity and multiple testing.ResultsThree transcriptionally distinct malignant T-cell (MTC) subtypes were identified: central memory-like (MTC CM), effector/effector memory-like (MTC E/EM) and regulatory-like (MTC Reg), each with unique expression programmes and putative vulnerabilities. MTC CM, the dominant population, expressed central memory markers, Th2/Th22 skewing and a broader KIR repertoire (KIR3DL2, KIR3DL1, KIR2DL3) than previously reported. MTC E/EM and MTC Reg were enriched for NR4A1 and ITGB1, respectively. Cell-cell communication analysis revealed KIR-MHC-I signalling and suggested that MTC-derived TNF and IL-10 may drive JAK/STAT pathway activity in non-T cells, indicating an underrecognized dimension of tumour-induced immune reprogramming.ConclusionsThis is one of the most comprehensive single-cell studies of leukaemic CTCL to date. We uncover new malignant T-cell states, broaden the known repertoire of KIR expression, and propose mechanisms of immune evasion that may contribute to treatment resistance. These findings lay the groundwork for subtype-specific and microenvironment-informed therapeutic strategies in Sézary syndrome, with potential implications for guiding targeted therapy selection but require validation in larger, independent cohorts.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41773808 · DOI · PMC13505662
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC) [5]
- Research article Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer [6]
- Research article European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2017 [7]
- Research article EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome [8]
- Research article Cutaneous T cell lymphoma: mycosis fungoides, Sézary syndrome, and other variants [9]
- Research article Mycosis fungoides and Sézary syndrome [10]
References
- Ruggiero E, Nicolay JP, Fronza R, Arens A, Paruzynski A, Nowrouzi A, et al.. High-resolution analysis of the human T-cell receptor repertoire. . 2015. PMID 26324409 · DOI · PMC4569693
- Iżykowska K, Przybylski GK, Przybylski GK, Gand C, Braun FC, Grabarczyk P, et al.. Genetic rearrangements result in altered gene expression and novel fusion transcripts in Sézary syndrome. . 2017. PMID 28489605 · DOI · PMC5503638
- Cristofoletti C, Bresin A, Picozza M, Picchio MC, Monzo F, Helmer Citterich M, et al.. Blood and skin-derived Sezary cells: differences in proliferation-index, activation of PI3K/AKT/mTORC1 pathway and its prognostic relevance. . 2019. PMID 30518812 · DOI · PMC6756225
- Childs BA, Elghonaimy EA, Ramakrishnan Geethakumari P, Kumar KA, Merola JF, Goff HW, et al.. Mapping malignant T-cell states and immune circuits in Sézary syndrome by single-cell analysis. . 2026. PMID 41773808 · DOI · PMC13505662
- Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al.. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007. PMID 17540844 · DOI
- Olsen EA, Whittaker S, Kim YH, Duvic M, Prince HM, Lessin SR, et al.. Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol. 2011. PMID 21576639 · DOI · PMC3422534
- Trautinger F, Eder J, Assaf C, Bagot M, Cozzio A, Dummer R, et al.. European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2017. Eur J Cancer. 2017. PMID 28365528 · DOI
- Trautinger F, Knobler R, Willemze R, Peris K, Stadler R, Laroche L, et al.. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer. 2006. PMID 16574401 · DOI
- Edelson RL. Cutaneous T cell lymphoma: mycosis fungoides, Sézary syndrome, and other variants. J Am Acad Dermatol. 1980. PMID 6988470 · DOI
- Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. 2008. PMID 18342689 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.