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 (810 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 chronic, malignant T-cell lymphoma of the skin. In the late stages, the lymph nodes and viscera are affected.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Where it fits in the MeSH classification
- Broader topics
- Lymphoma, T-Cell, Cutaneous
- Narrower topics
- Pagetoid Reticulosis
Related concepts
Research landscape
PubMed indexes 6,582 records tagged with the MeSH heading “Mycosis Fungoides” (counts retrieved 2026-10-29). 1,092 of them (16.6%) were published from 2020 onward, and the busiest period so far is through 1999 with 3,024 records.
Reviews account for 785 records (11.9%) and meta-analyses for 16. Randomized controlled trials number 35 (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 | 3,024 |
| 2000-2009 | 1,051 |
| 2010-2019 | 1,415 |
| 2020 onward | 1,092 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 785 | 11.9% |
| Meta-analyses | 16 | 0.2% |
| Randomized controlled trials | 35 | 0.5% |
Method: PubMed query "Mycosis Fungoides"[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 |
|---|---|---|---|---|
| Plaque stage folliculotropic mycosis fungoides: histopathologic features and prognostic factors in a series of 40 patients | 2020 | Research article Design not recorded in structured metadata | 23 | cc by PMID 31755567 · DOI · PMC7028105 |
| Understanding Cell Lines, Patient-Derived Xenograft and Genetically Engineered Mouse Models Used to Study Cutaneous T-Cell Lymphoma | 2022 | Review article Design not recorded in structured metadata | 18 | cc by PMID 35203244 · DOI · PMC8870189 |
| CD147-Cyclophilin a Interactions Promote Proliferation and Survival of Cutaneous T-Cell Lymphoma | 2021 | Research article Design not recorded in structured metadata | 18 | cc by PMID 34360654 · DOI · PMC8346093 |
| 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 |
Plaque stage folliculotropic mycosis fungoides: histopathologic features and prognostic factors in a series of 40 patients [1]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundFolliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides.
- Closing excerpt
- It further shows that histological examination is a valuable tool to differentiate between indolent and aggressive disease.
- 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 (179 words)
BackgroundFolliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides. Recent studies recognized indolent and aggressive subgroups of FMF, but there is controversy how patients presenting with plaques should be classified. The present study describes the histopathologic features of 40 FMF plaques. The aim of the study was to identify risk factors for disease progression and poor outcome in this group.MethodsClinical, histopathological, and immunophenotypical data from 40 patients with plaque stage FMF were reviewed and analysed for risk factors for disease progression and survival.ResultsAfter a median follow-up of 80 months, disease progression occurred in 20 of 40 patients. Percentage of atypical cells, cell size, percentage of Ki-67+ cells, and co-existent interfollicular epidermotropism, but not the extent of perifollicular infiltrates, were associated with disease progression and reduced survival, while extensive follicular mucinosis was associated with increased survival.ConclusionsThis study underlines that FMF patients presenting with plaques represent a heterogeneous group and that a subgroup of these patients may have an indolent clinical course. It further shows that histological examination is a valuable tool to differentiate between indolent and aggressive disease.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 31755567 · DOI · PMC7028105
Understanding Cell Lines, Patient-Derived Xenograft and Genetically Engineered Mouse Models Used to Study Cutaneous T-Cell Lymphoma [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Cutaneous T cell lymphoma (CTCL) is a spectrum of lymphoproliferative disorders caused by the infiltration of malignant T cells into the skin.
- Closing excerpt
- The current review summarizes cell lines and xenograft/GEMMs used to study and understand the etiology and heterogeneity of CTCL.
- 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 (163 words)
Cutaneous T cell lymphoma (CTCL) is a spectrum of lymphoproliferative disorders caused by the infiltration of malignant T cells into the skin. The most common variants of CTCL include mycosis fungoides (MF), Sézary syndrome (SS) and CD30+ Lymphoproliferative disorders (CD30+ LPDs). CD30+ LPDs include primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP) and borderline CD30+ LPD. The frequency of MF, SS and CD30+ LPDs is ~40-50%, + LPDs. Molecular analysis of the other two frequently used Human T-Cell Lymphotropic Virus-1 (HTLV-1)+ cell lines, MJ and Hut102, were found to have characteristics of Adult T-cell Leukemia/Lymphoma (ATLL). Studies in mouse models demonstrated that xenograft tumors could be grown using MyLa, HH, H9, Hut78, PB2B and SZ4 cells in NSG (NOD Scid gamma mouse) mice, while several additional experimental GEMMs were established to study the pathogenesis, effect of drugs and inflammatory cytokines in CTCL. The current review summarizes cell lines and xenograft/GEMMs used to study and understand the etiology and heterogeneity of CTCL.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 35203244 · DOI · PMC8870189
CD147-Cyclophilin a Interactions Promote Proliferation and Survival of Cutaneous T-Cell Lymphoma [3]
Abstract reading summary — source excerpts
- Opening excerpt
- CD147, a transmembrane glycoprotein that belongs to the immunoglobulin superfamily, and cyclophilin A (CypA), one of the binding partners of CD147, are overexpressed in tumor cells and associated with the progression of several malignancies, including both solid and hematological malignancies.
- Closing excerpt
- These results suggest that CD147-CypA interactions can contribute to the proliferation of MF/SS tumor cells in both a autocrine and paracrine manner, and that the disruption of CD147-CypA interactions could be a new therapeutic strategy for the treatment of MF/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 (183 words)
CD147, a transmembrane glycoprotein that belongs to the immunoglobulin superfamily, and cyclophilin A (CypA), one of the binding partners of CD147, are overexpressed in tumor cells and associated with the progression of several malignancies, including both solid and hematological malignancies. However, CD147 and CypA involvement in cutaneous T-cell lymphoma (CTCL) has not been reported. In this study, we examined CD147 and CypA expression and function using clinical samples of mycosis fungoides (MF) and Sézary syndrome (SS) and CTCL cell lines. CD147 and CypA were overexpressed by tumor cells of MF/SS, and CypA was also expressed by epidermal keratinocytes in MF/SS lesional skin. Serum CypA levels were increased and correlated with disease severity markers in MF/SS patients. Anti-CD147 antibody and/or anti-CypA antibody suppressed the proliferation of CTCL cell lines, both in vitro and in vivo, via downregulation of phosphorylated extracellular-regulated kinase 1/2 and Akt. These results suggest that CD147-CypA interactions can contribute to the proliferation of MF/SS tumor cells in both a autocrine and paracrine manner, and that the disruption of CD147-CypA interactions could be a new therapeutic strategy for the treatment of MF/SS.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 34360654 · DOI · PMC8346093
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 Defining early mycosis fungoides [7]
- Research article The pathogenesis of mycosis fungoides [8]
- Research article Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers [9]
- Research article Mycosis fungoides and Sézary syndrome [10]
References
- van Santen S, Jansen PM, Quint KD, Vermeer MH, Willemze R. Plaque stage folliculotropic mycosis fungoides: histopathologic features and prognostic factors in a series of 40 patients. . 2020. PMID 31755567 · DOI · PMC7028105
- Gill RPK, Gantchev J, Martínez Villarreal A, Ramchatesingh B, Netchiporouk E, Akilov OE, et al.. Understanding Cell Lines, Patient-Derived Xenograft and Genetically Engineered Mouse Models Used to Study Cutaneous T-Cell Lymphoma. . 2022. PMID 35203244 · DOI · PMC8870189
- Sakamoto M, Miyagaki T, Kamijo H, Oka T, Boki H, Takahashi-Shishido N, et al.. CD147-Cyclophilin a Interactions Promote Proliferation and Survival of Cutaneous T-Cell Lymphoma. . 2021. PMID 34360654 · DOI · PMC8346093
- 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
- Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al.. Defining early mycosis fungoides. J Am Acad Dermatol. 2005. PMID 16310068 · DOI
- Girardi M, Heald PW, Wilson LD. The pathogenesis of mycosis fungoides. N Engl J Med. 2004. PMID 15128898 · DOI
- Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014. PMID 24438969 · 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.