Raras
Buscar doenças, sintomas, genes...
Síndrome Sezary
ORPHA:3162CID-10 · C84.1CID-11 · 2B02DOENÇA RARA

A Síndrome de Sézary (SS) é uma forma agressiva de linfoma de células T que afeta a pele. Ela é caracterizada por três sinais principais: uma vermelhidão intensa e generalizada na pele (eritrodermia), inchaço dos gânglios linfáticos e a presença de linfócitos atípicos circulando no sangue (chamados de células de Sézary).

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Síndrome de Sézary (SS) é uma forma agressiva de linfoma de células T que afeta a pele. Ela é caracterizada por três sinais principais: uma vermelhidão intensa e generalizada na pele (eritrodermia), inchaço dos gânglios linfáticos e a presença de linfócitos atípicos circulando no sangue (chamados de células de Sézary).

Pesquisas ativas
10 ensaios
33 total registrados no ClinicalTrials.gov
Publicações científicas
2.628 artigos
Último publicado: 2026 Apr 16

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C84.1
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧬
Pele e cabelo
5 sintomas
🩸
Sangue
3 sintomas
🫃
Digestivo
2 sintomas
💪
Músculos
2 sintomas
🛡️
Imunológico
2 sintomas
🧠
Neurológico
2 sintomas

+ 13 sintomas em outras categorias

Características mais comuns

90%prev.
Linfoma cutâneo de células T
Muito frequente (99-80%)
90%prev.
Linfadenopatia
Muito frequente (99-80%)
90%prev.
Neoplasia da pele
Muito frequente (99-80%)
90%prev.
Pele seca
Muito frequente (99-80%)
90%prev.
Prurido
Muito frequente (99-80%)
90%prev.
Morfologia anormal de linfócitos T
Muito frequente (99-80%)
29sintomas
Muito frequente (10)
Frequente (7)
Ocasional (12)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 29 características clínicas mais associadas, ordenadas por frequência.

Linfoma cutâneo de células TCutaneous T-cell lymphoma
Muito frequente (99-80%)90%
LinfadenopatiaLymphadenopathy
Muito frequente (99-80%)90%
Neoplasia da peleNeoplasm of the skin
Muito frequente (99-80%)90%
Pele secaDry skin
Muito frequente (99-80%)90%
PruridoPruritus
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.628PubMed
Últimos 10 anos200publicações
Pico2025115 papers
Linha do tempo
2026Hoje · 2026🧪 2000Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

3 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Not applicable.

TNFRSF1BTumor necrosis factor receptor superfamily member 1BDisease-causing somatic mutation(s) inModerado
FUNÇÃO

Receptor with high affinity for TNFSF2/TNF and approximately 5-fold lower affinity for homotrimeric TNFSF1/lymphotoxin-alpha. The TRAF1/TRAF2 complex recruits the apoptotic suppressors BIRC2 and BIRC3 to TNFRSF1B/TNFR2. This receptor mediates most of the metabolic effects of TNF. Isoform 2 blocks TNF-induced apoptosis, which suggests that it regulates TNF function by antagonizing its biological activity

LOCALIZAÇÃO

Cell membraneSecreted

VIAS BIOLÓGICAS (4)
Neutrophil degranulationTNFR2 non-canonical NF-kB pathwayTNFs bind their physiological receptorsInterleukin-4 and Interleukin-13 signaling
EXPRESSÃO TECIDUAL(Ubíquo)
Sangue
474.3 TPM
Baço
180.8 TPM
Tecido adiposo
110.3 TPM
Linfócitos
105.9 TPM
Pulmão
100.4 TPM
OUTRAS DOENÇAS (2)
mycosis fungoidesSezary syndrome
HGNC:11917UniProt:P20333
CD28T-cell-specific surface glycoprotein CD28Part of a fusion gene inRestrito
FUNÇÃO

Receptor that plays a role in T-cell activation, proliferation, survival and the maintenance of immune homeostasis (PubMed:1650475, PubMed:7568038). Functions not only as an amplifier of TCR signals but delivers unique signals that control intracellular biochemical events that alter the gene expression program of T-cells (PubMed:24665965). Stimulation upon engagement of its cognate ligands CD80 or CD86 increases proliferation and expression of various cytokines in particular IL2 production in bo

LOCALIZAÇÃO

Cell membraneCell surface

VIAS BIOLÓGICAS (2)
Co-stimulation by CD28Nef mediated downregulation of CD28 cell surface expression
MECANISMO DE DOENÇA

Immunodeficiency 123 with HPV-related verrucosis

An autosomal recessive immunologic disorder characterized by susceptibility to human papilloma virus (HPV) infections and the development of HPV-related common verrucosis in the first decade of life. In some patients with HPV2 infection, warts may progress to severe generalized hyperkeratotic cutaneous papillomatosis with cutaneous horns ('tree-man' phenotype). In patients with HPV4 infection, warts remains stable and may even regress with age.

OUTRAS DOENÇAS (3)
immunodeficiency 123 with HPV-related verrucosismycosis fungoidesSezary syndrome
HGNC:1653UniProt:P10747
CTLA4Cytotoxic T-lymphocyte protein 4Part of a fusion gene inAltamente restrito
FUNÇÃO

Inhibitory receptor acting as a major negative regulator of T-cell responses (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:18641304, PubMed:28484017). Acts as a decoy receptor: the affinity of CTLA4 for its natural B7 family ligands, CD80 and CD86, is considerably stronger than the affinity of their cognate stimulatory coreceptor CD28 (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:28484017)

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (3)
Co-stimulation by CD28Co-inhibition by CTLA4RUNX1 and FOXP3 control the development of regulatory T lymphocytes (Tregs)
MECANISMO DE DOENÇA

Systemic lupus erythematosus

A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.

EXPRESSÃO TECIDUAL(Tecido-específico)
Baço
5.8 TPM
Intestino delgado
4.9 TPM
Pulmão
4.3 TPM
Testículo
2.5 TPM
Sangue
1.8 TPM
OUTRAS DOENÇAS (6)
autoimmune lymphoproliferative syndrome due to CTLA4 haploinsufficiencysystemic lupus erythematosusgranulomatosis with polyangiitismycosis fungoides
HGNC:2505UniProt:P16410

Variantes genéticas (ClinVar)

191 variantes patogênicas registradas no ClinVar.

🧬 CTLA4: NM_005214.5(CTLA4):c.148_165del (p.Ser50_Ser55del) ()
🧬 CTLA4: NM_005214.5(CTLA4):c.174_175del (p.Cys58_Glu59delinsTer) ()
🧬 CTLA4: NM_005214.5(CTLA4):c.123_124delinsTT (p.Gln42Ter) ()
🧬 CTLA4: NM_005214.5(CTLA4):c.313G>A (p.Gly105Ser) ()
🧬 CTLA4: NM_005214.5(CTLA4):c.406C>G (p.Pro136Ala) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 1 variantes classificadas pelo ClinVar.

1
Patogênica (100.0%)
VARIANTES MAIS SIGNIFICATIVAS
BCL10: NM_003921.5(BCL10):c.428del (p.Phe143fs) [Pathogenic]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
2Fase 27
1Fase 13
·Pré-clínico8
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 19 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Sezary

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

33 ensaios clínicos encontrados, 10 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
1.111 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.111

#1

Inducible Costimulator and Its Ligand Promote Proliferation and Migration of Tumor Cells in Cutaneous T-Cell Lymphoma.

International journal of molecular sciences2026 Jan 30

Inducible costimulator (ICOS) is a costimulatory immune checkpoint receptor expressed on activated T-cells, while the ICOS ligand (ICOSL) is expressed on antigen-presenting cells. The ICOS-ICOSL axis promotes the survival of memory and effector T-cells and induces several immune responses. In addition, the ICOS-ICOSL interaction induces cell proliferation, cell survival, and cytokine production. The roles of ICOS and ICOSL in cutaneous T-cell lymphoma (CTCL) are unclear. In this study, we examined the roles of ICOS and ICOSL in CTCL. The tumor cells co-expressed ICOS and ICOSL, and the upregulated expression of ICOS and ICOSL reflected disease severity. Anti-ICOS and anti-ICOSL neutralizing antibodies inhibited both the in vitro and in vivo proliferation of CTCL cell lines. The anti-ICOSL neutralizing antibodies induced apoptosis and suppressed CCR4 expression on tumor cells, inhibiting CCR4-CCL17-mediated migration. These results suggest that the ICOS-ICOSL axis plays an essential role in CTCL pathogenesis, and targeting the ICOS-ICOSL axis could be a viable strategy for treating CTCL.

#2

Resminostat for maintenance treatment in patients with advanced-stage mycosis fungoides or Sézary syndrome: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.

The Lancet. Haematology2026 Feb

In advanced-stage cutaneous T-cell lymphoma (CTCL), current therapeutic options rarely provide long-lasting responses. We aimed to evaluate the efficacy and safety of a histone deacetylase inhibitor, resminostat, as maintenance therapy in patients with advanced-stage mycosis fungoides or Sézary syndrome, in whom disease control had been previously met. We conducted a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial (RESMAIN) at 55 medical centres in Austria, Belgium, France, Germany, Greece, Italy, the Netherlands, Poland, Spain, Switzerland, the UK, and Japan. Adult patients (aged ≥18 years) with histologically confirmed, stage IIB-IVB mycosis fungoides or Sézary syndrome; an Eastern Cooperative Oncology Group performance status score of 0-2; and disease control after at least one previous systemic therapy or total skin electron beam were eligible for inclusion. Patients were randomly assigned to receive either oral resminostat (600 mg) or matching oral placebo once daily for 5 days, followed by a treatment-free period of 9 days, within a 14-day treatment cycle. Randomisation was stratified by disease stage (IIB-IVA1 vs IVA2-IVB) and remission status following previous therapy (complete or partial response vs stable disease) by use of a dynamic block allocation process (block size 100 patients). Participants, investigators, site staff, and study personnel involved in outcome assessment and data analysis were masked to group assignment. Patients with disease progression during masked treatment were unmasked; patients on placebo were offered open-label resminostat. Treatment was continued until disease progression or unacceptable toxicity. The primary endpoint was progression-free survival, defined as the time from randomisation to disease progression or death from any cause (whichever occurred first), analysed by intention to treat. This trial is registered with ClincalTrials.gov (NCT02953301) and has been completed. Between Jan 9, 2017, and May 11, 2022, 234 patients were screened for eligibility, of whom 201 (86%) patients were randomly assigned: 100 (50%) to resminostat and 101 (50%) to placebo. 123 (61%) participants were men and 78 (39%) were women, with a median age of 64 years (range 30-87). Most participants (173 [86%]) were White, 19 (9%) were Asian (mainly Japanese), two (1%) were Black, and seven (3%) were either another race or ethnicity, or did not disclose these data. Median progression-free survival was 8·3 months (95% CI 4·2-15·7) in the resminostat group and 4·2 months (2·8-6·4) in the placebo group (HR 0·62 [95% CI 0·42-0·92]; p=0·015). Median follow-up time for progression-free survival was 11·2 months (95% CI 5·6-19·6) in the resminostat group and 17·0 months (13·9-30·5) in the placebo group. Adverse events were reported in 96 (96%) patients receiving resminostat and in 81 (80%) patients receiving placebo. Serious adverse events occurred in 19 (19%) patients in the resminostat group, 11 (11%) of which were considered related to treatment, and in 12 (12%) in the placebo group, of which one (1%) was considered to be treatment-related. Adverse events of grade 3 or above occurred in 38 (38%) patients in the resminostat group and in 15 (15%) patients in the placebo group. The most common treatment-related adverse events were nausea (68 [68%] in the resminostat group vs six [6%] in the placebo group), diarrhoea (44 [44%] vs nine [9%]), vomiting (32 [32%] vs one [1%]), and fatigue (29 [29%] vs 14 [14%]). There were no treatment-related deaths. These findings support the beneficial effect of resminostat maintenance therapy in patients with advanced CTCL. The overall safety profile of resminostat was acceptable, with gastrointestinal side-effects occurring most frequently. Anti-emetic prophylaxis should be considered in the future to manage side-effects and to improve tolerability and adherence to maintenance therapy. 4SC AG.

#3

Protocol for a mixed-methods modified Delphi study for the development of a core domain set to assess the health-related quality of life of patients with mycosis fungoides and Sézary syndrome in clinical trials.

BMJ open2026 Feb 06

Cutaneous T cell lymphoma (CTCL) is a group of non-Hodgkin lymphomas that primarily affects the skin and can mimic inflammatory dermatoses. Unlike many skin diseases, CTCL can lead to disabling symptoms, and advanced CTCL can even be fatal. Early studies investigating health-related quality of life (HRQOL) in patients with mycosis fungoides (MF) and Sézary syndrome (SS), common subtypes of CTCL, demonstrated significant impairment across numerous domains. The aim of this current study is to develop a core domain set (CDS) to identify the essential aspects of MF/SS that influence HRQOL that should be measured in therapeutic clinical trials. In the future, this set of core concepts will be used to identify the best patient- reported outcome measure(s) (PROM) for HRQOL for MF/SS clinical research. Multiple strategies will be used to generate candidate concepts: systematic review of the literature, qualitative study and a survey study of healthcare providers. A Delphi consensus process including a comprehensive group of stakeholders (patients, caregivers/care partners, a multidisciplinary group of healthcare professionals, patient advocacy groups, pharmaceutical industry representatives, methodologists and government agencies) will be used to achieve consensus. Statistical corrections for multiple significance testing and false positive findings will be undertaken. The study was submitted for and received institutional review board approval at the University of Washington (IRB# STUDY00018890 and STUDY00019407). Informed consent will be obtained from all participants where necessary. We will disseminate our findings through peer-reviewed, open access publications and presentations at national/international conferences. We will provide a plain language summary in lay terms for patients and families to patient advocacy groups for distribution to their network. The protocol is registered in the Core Outcome Measures in Effectiveness Trials (COMET) database.

#4

T-Cell Receptor Clonotypes and Aggressive Subtypes in Cutaneous T-Cell Lymphoma.

JAMA dermatology2026 Jan 01

T-cell receptor (TCR) clonotype patterns across disease stages and histologic subtypes in mycosis fungoides (MF) and Sézary syndrome (SS) remain poorly characterized, limiting their use in risk stratification. To assess the association of TCR β (TCRB) and γ (TCRG) clonotypes with disease stage, folliculotropism, large-cell transformation, and overall survival (OS) as well as clonal abundance (percentage of total reads) with immune checkpoint expression. This retrospective cohort study conducted at City of Hope (Duarte, California) included patients with stage IA to IVB MF/SS who underwent TCR next-generation sequencing on lesional skin biopsy specimens collected between June 2020 to October 2024; duplicate samples were excluded. Analyses were performed from November 2024 to April 2025. Associations between clinical and genetic categorical variables were evaluated using the Fisher exact test. OS was analyzed using Kaplan-Meier estimates, with univariate and multivariable models applied to assess prognostic factors. Of the 125 patients (42 female [33.6%] and 74 male individuals [66.4%]; mean [SD] age, 62.4 [15.9] years) who underwent TCR sequencing, at least 1 clonal TCRB and/or TCRG gene segment was identified in 98 patients (78%). Clonal TCRB and TCRG segments were detected in 72 (57.6%) and 92 patients (73.6%), respectively. The clonal Vb20 segment was significantly associated with folliculotropism and concurrent large-cell transformation compared with classic MF/SS (7 of 17 [41%] vs 0 of 30 [0%]; P < .001), marginally significantly associated with advanced-stage MF/SS compared with early-stage MF (8 of 38 [21%] vs 0 of 34 [0%]; P = .01). Clonal Vg8 was significantly associated with advanced-stage MF/SS compared with early-stage MF (25 of 53 [47%] vs 8 of 39 [21%]; P = .01) and correlated with poorer OS. Additionally, the higher percentage of total reads for TCRG was positively correlated with increased expression of immune checkpoints programmed cell death 1 and inducible T-cell costimulator but not with programmed cell death ligand 1. This cohort study's analysis of TCRB and TCRG repertoires identified specific clonotypes that were associated with more aggressive subtypes and poorer survival in patients with MF/SS. Incorporating TCR sequencing into clinical practice may enhance risk stratification, enabling earlier identification of high-risk patients who could benefit from closer monitoring and timely implementation of more intensive treatment strategies in the disease course to improve clinical outcomes.

#5

Safety of Concurrent Systemic Therapy and Total Skin Electron Therapy in Cutaneous T-Cell Lymphoma.

Clinical lymphoma, myeloma &amp; leukemia2026 Mar 01

To evaluate the safety and outcomes of total skin electron therapy (TSET) delivered with concurrent systemic therapy (CS) for cutaneous T-cell lymphoma (CTCL). We retrospectively identified CTCL patients (including Sézary syndrome and mycosis fungoides) receiving TSET from 2013 to 2023. Acute toxicities were graded per CTCAE v5.0 and defined as new or worsened (N/W) from baseline. Cutaneous response was assessed at 2-8 weeks and 6 months post-TSET. Analyses of toxicity and response were performed per TSET course and classified as CS if any systemic agent was administered during treatment. Survival outcomes were estimated using Kaplan-Meier methods in per-patient analyses using one index course per patient, with time measured from the start of TSET. A total of 121 TSET courses were analyzed, including 75 (62%) with CS and 46 (38%) without concurrent systemic therapy (NCS). Median follow-up was 21 months overall and longer for CS courses (28 vs. 16 months; P = .019). Nearly all courses experienced ≥1 N/W toxicity, most of which was low grade. High-grade toxicities occurred in 13% of CS and 26% of NCS courses (P = .080), with no grade ≥4 events. Overall, toxicity burden and symptom outcomes were similar between groups. Toxicity severity appeared comparable across commonly used systemic therapy classes; one patient receiving concurrent methotrexate experienced disproportionate toxicity. Early overall response rates at 2-8 weeks were 90% (CS) and 92% (NCS) (P = .138). In per-patient analyses, median progression-free survival was 8.61 versus 6.08 months (log-rank P = .325), and 24-month overall survival was 85.7% versus 68.3% (log-rank P = .098) for CS and NCS, respectively. TSET can be delivered safely with most commonly used CS therapies in CTCL.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.318 artigos no totalmostrando 196

2026

Safety of Concurrent Systemic Therapy and Total Skin Electron Therapy in Cutaneous T-Cell Lymphoma.

Clinical lymphoma, myeloma &amp; leukemia
2026

Mogamulizumab-associated lymphadenopathy masquerading as lymphoma progression.

JAAD case reports
2026

Leonine Facies in Sézary Syndrome.

Journal of cutaneous medicine and surgery
2026

Cutaneous T-cell lymphomas and dupilumab for atopic dermatitis: A systematic review and expert consensus.

Journal of the European Academy of Dermatology and Venereology : JEADV
2026

Mycosis fungoides/Sézary syndrome and systemic Janus kinase inhibitors: a real-world retrospective study on behalf of the EORTC-CLTG.

The British journal of dermatology
2026

Mapping malignant T-cell states and immune circuits in Sézary syndrome by single-cell analysis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2026

Persistence of sex differences and stable trends in mortality in patients with mycosis fungoides and Sézary syndrome in Spain (1983-2022).

Medicina clinica
2026

Mycosis fungoides and Sézary syndrome.

British journal of haematology
2026

Type 2 diabetes and risk of non-Hodgkin lymphoma and multiple myeloma: a pooled analysis.

JNCI cancer spectrum
2026

Inducible Costimulator and Its Ligand Promote Proliferation and Migration of Tumor Cells in Cutaneous T-Cell Lymphoma.

International journal of molecular sciences
2026

Laboratory Evaluation of Peripheral Blood Involvement in Mycosis Fungoides and Sézary Syndrome: Evolution of Flow Cytometry and Morphology Quantification and Interpretation.

Cancers
2025

Efficacy and tolerability of mogamulizumab in mycosis fungoides and Sézary Syndrome: a monocentric retrospective study.

Frontiers in oncology
2026

Resminostat for maintenance treatment in patients with advanced-stage mycosis fungoides or Sézary syndrome: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.

The Lancet. Haematology
2026

Protocol for a mixed-methods modified Delphi study for the development of a core domain set to assess the health-related quality of life of patients with mycosis fungoides and Sézary syndrome in clinical trials.

BMJ open
2026

A historical review of mycosis fungoides: from Alibert to mogamulizumab.

Skin health and disease
2026

Diverse transcriptomic and mutational patterns but limited functional pathway alterations in patient-derived Sézary syndrome cells.

The Journal of investigative dermatology
2026

Racial Disparities in United States Clinical Trial Enrollment for Mycosis Fungoides and Sézary Syndrome.

Journal of drugs in dermatology : JDD
2026

Sézary syndrome arising from cutaneous epitheliotropic T-cell lymphoma, resembling human folliculotropic mycosis fungoides, in a dog.

Journal of comparative pathology
2026

Diagnostic Implications of Dupilumab-Refractory Atopic Dermatitis: Risk of Underlying Cutaneous T-Cell Lymphoma and the Role of Skin Biopsy.

International journal of dermatology
2025

Neutrophil-Galectin-9 Axis Linking Innate and Adaptive Immunity in ATL, Sézary Syndrome, COVID-19, and Psoriasis: An AI-Assisted Integrative Review.

Reports (MDPI)
2026

How I manage mycosis fungoides and Sézary syndrome: Current controversies and unmet needs.

British journal of haematology
2025

Novel isochromosome 7q and NOTCH1 mutation in an 88-year-old male with Sézary syndrome.

JAAD case reports
2026

Sézary syndrome presenting as vitiligo-like leukoderma with response and repigmentation to mogamulizumab and extracorporeal photopheresis.

JAAD case reports
2026

Delayed Diagnosis of Sézary Syndrome: Lessons From a Psoriasiform Presentation.

Clinical case reports
2025

Brentuximab Vedotin in Advanced-Stage Mycosis Fungoides/Sézary Syndrome with Low CD30 Expression: Real-World Data from the German Cutaneous Lymphoma Network.

Cancers
2026

Incidence and Mortality of Mycosis Fungoides and Sezary Syndrome: A Nationwide Registry Study in Finland.

Acta dermato-venereologica
2026

Mogamulizumab plus etoposide in the management of mycosis fungoides with blood involvement: a case report.

Therapeutic advances in hematology
2025

Initial experience with a novel device for supporting a debilitated patient during total skin electron beam therapy.

Journal of cancer research and therapeutics
2025

[18F]FDG PET/CT revealing extensive cutaneous involvement in Sézary syndrome.

Nuclear medicine review. Central &amp; Eastern Europe
2025

Case Report: Dose-dependent response to oclacitinib in a dog with Sézary syndrome.

Frontiers in veterinary science
2025

Translation, Cross-cultural Adaptation, Correlation With Other PROMs and Validation of the MF/SS-CTCL-QoL Questionnaire on Quality of Life in Mycosis Fungoides (MF) and Sézary Syndrome (SS).

Actas dermo-sifiliograficas
2026

Relapsed/refractory mycosis fungoides and Sezary syndrome: biology, clinical manifestations and therapeutic options.

Leukemia &amp; lymphoma
2026

Clinical Implications of the Skin Microbiota in the Therapy of Cutaneous T Cell Lymphoma: A Scoping Review.

Dermatology and therapy
2026

Recent trends of incidence and mortality of cutaneous lymphomas in Germany.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2026

Disparities in immunotherapy treatment for mycosis fungoides and Sézary syndrome in the National Cancer Database.

Journal of the American Academy of Dermatology
2025

Unusual presentation of fungal keratitis and endogenous endophthalmitis in Sézary syndrome: a case report.

American journal of ophthalmology case reports
2025

Retrospective Analysis of Second Primary Malignancies in Patients with Mycosis Fungoides and Sézary Syndrome in Croatia.

Dermatology practical &amp; conceptual
2025

OX-40 signaling promotes tumorigenesis in CTCL by regulating ERK activation.

Frontiers in immunology
2025

scRNA-Seq reveals anti-lymphoma immune responses in mogamulizumab-associated skin eruptions.

Journal of the European Academy of Dermatology and Venereology : JEADV
2026

IL4/IL13 Inhibition via Dupilumab Reduces Malignant T-cell Proliferation and Promotes Antitumor Immunity in Sézary Syndrome.

Cancer immunology research
2025

The Evaluation of Skin Infiltration in Mycosis Fungoides/Sézary Syndrome Using the High-Frequency Ultrasonography.

Journal of clinical medicine
2026

T-Cell Receptor Clonotypes and Aggressive Subtypes in Cutaneous T-Cell Lymphoma.

JAMA dermatology
2025

A rare case of Sézary syndrome - A diagnostic challenge.

Indian journal of dermatology, venereology and leprology
2025

Extracorporeal photopheresis therapy rapidly changes the cytokine profile and tumor microenvironment in cutaneous T cell lymphoma.

Frontiers in immunology
2025

Co-expression of CD30 and SLFN11 serves as a dual biomarker for the treatment of cutaneous T-cell lymphoma.

NAR cancer
2025

Lymph node detection of monoclonal T-cell population correlates with poorer prognosis in mycosis fungoides and Sèzary syndrome.

British journal of haematology
2026

Dual TRBC1-CD3 Immunohistochemistry Shows High Diagnostic Utility in Differentiating Mycosis Fungoides/Sezary Syndrome From Reactive Inflammatory Dermatoses in Skin Biopsies.

The American journal of surgical pathology
2025

Allogeneic hematopoietic cell transplant in cutaneous T-cell lymphomas: recommendations from the EBMT PH&G Committee.

Bone marrow transplantation
2026

Racial differences in mycosis fungoides and Sézary syndrome: A multicenter cohort study.

Journal of the American Academy of Dermatology
2026

Allogeneic haematopoietic cell transplant in cutaneous T-cell lymphomas: Recommendations from the EBMT PH&G Committee.

Journal of the European Academy of Dermatology and Venereology : JEADV
2025

Expanded CD4+CD57+ T-large granular lymphocytes: A diagnostic pitfall in blood staging of mycosis fungoides/Sézary syndrome.

American journal of clinical pathology
2025

A to F Think MF! A memory aid for early recognition of mycosis fungoides/Sézary syndrome.

The British journal of dermatology
2025

Weekly dosing schedule of brentuximab vedotin is well tolerated in mycosis fungoides/Sézary syndrome.

British journal of haematology
2026

Sézary syndrome as an unusual cause of Loeffler endocarditis.

Revista espanola de cardiologia (English ed.)
2025

Mogamulizumab-associated rash and lymphocytopenia coinciding with treatment response of Sézary syndrome.

European journal of dermatology : EJD
2025

Quality and duration of responses with mogamulizumab in cutaneous T-cell lymphomas: Insights into long-lasting outcomes.

British journal of haematology
2025

An Update on Single-Cell RNA Sequencing in Illuminating Disease Mechanisms of Cutaneous T-Cell Lymphoma.

Cancers
2025

Targeted Radiotherapy in Primary Cutaneous Lymphomas: Precision, Efficacy, and Evolving Strategies.

Cancers
2025

Recurrence of mogamulizumab-associated rash in patients with relapsed erythrodermic cutaneous T-cell lymphoma after retreatment with mogamulizumab.

JAAD case reports
2025

Skin Response to Mogamulizumab in Patients With Mycosis Fungoides and Sezary Syndrome: A Retrospective, Real World Cohort.

Clinical lymphoma, myeloma &amp; leukemia
2025

Economic burden and health-related quality of life in Chinese patients with mycosis fungoides and Sézary syndrome.

Cancer pathogenesis and therapy
2025

Expressed mutated genes in Sezary syndrome and their potential prognostic value in patients treated with extracorporeal photopheresis.

Frontiers in immunology
2025

Synergistic Efficacy of Chidamide and LB100 in Sézary Syndrome via TNC Downregulation and PI3K/AKT/mTOR Dephosphorylation.

Cancer science
2025

High-Throughput Sequencing and Estimated Relapse Risk in Patients With Sézary Syndrome.

JAMA dermatology
2025

Janus kinase inhibitors - a role for the treatment of cutaneous T-cell lymphomas?

Oncology reviews
2025

Unraveling the role of chemokines in cutaneous T-cell lymphoma: expression levels at different stages.

Frontiers in immunology
2026

Clinical and Prognostic Features of Erythrodermic Cutaneous T-Cell Lymphoma: A Retrospective Study of 35 Patients.

Turkish journal of haematology : official journal of Turkish Society of Haematology
2025

Low-dose total skin electron beam therapy in erythrodermic mycosis fungoides and Sézary syndrome: Results From the Prospective S-MISR Study.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Case report of non-gene editing CD7 CAR T cell therapy in CD7+ Sézary syndrome: preclinical validation and first-in-human use.

Frontiers in immunology
2026

CRISPR-Cas9 screen reveals that inhibition of enhancer of zeste homolog 2 sensitizes malignant T cells to dimethyl-fumarate-induced cell death.

The FEBS journal
2025

Pruritus Is an Indicator for Quality of Life in Cutaneous T-Cell Lymphoma.

The Journal of dermatology
2025

Psychosocial burden of patients with mycosis fungoides and Sézary syndrome compared to other dermatological patients. Pilot study.

Postepy dermatologii i alergologii
2025

A new prognostic index (CLIPI) for advanced cutaneous lymphoma enables precise patient risk stratification.

Blood
2025

Immune pathways, current and potential therapies in Mycosis fungoides and Sezary syndrome.

Expert review of clinical immunology
2025

Pharmacovigilance study for the identification of mogamulizumab-induced immune-related adverse events using a real-world database.

The oncologist
2025

New nonchemotherapy treatment options for cutaneous T-cell lymphomas an update.

Expert review of anticancer therapy
2025

Acneiform type of mogamulizumab-associated rash.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Linperlisib Plus Chidamide in Relapsed or Refractory Cutaneous T-Cell Lymphoma: A Nonrandomized Clinical Trial.

JAMA dermatology
2025

Coexistence of Mycosis Fungoides and Photosensitive or Autoimmune Diseases. The Therapeutic Challenge: A retrospective Case Series from a Tertiary Referral Center.

The Israel Medical Association journal : IMAJ
2025

Increased mortality due to lymphoma and infections in patients with mycosis fungoides or Sézary syndrome: a Swedish nationwide population-based cohort study.

The British journal of dermatology
2025

High Prevalence and Risk Factors for Infection with Human T-Lymphotropic Viruses 1 and 2 in the Municipality of Ananindeua, Pará, Northern Brazil.

Viruses
2025

CCR8 Immunohistochemistry Highlights CCR8 as a Diagnostic and Therapeutic Biomarker in Cutaneous T-Cell Lymphoma.

The Journal of investigative dermatology
2025

Structural Insights into the ADCC Mechanism and Resistance of Mogamulizumab, a First-in-Class Anti-CCR4 Therapy for Cutaneous T Cell Lymphoma.

International journal of molecular sciences
2025

Large-cell transformation of mycosis fungoides: Patterns of care and patient outcomes.

British journal of haematology
2025

Evaluating mogamulizumab in the treatment of primary cutaneous T-cell lymphoma.

Immunotherapy
2025

A phase 1 study of interleukin-15 in combination with mogamulizumab in relapsed and refractory T-cell malignancies.

Blood neoplasia
2025

Classical and biological treatments in mycosis fungoides/Sézary syndrome. New horizons in oncodermatology.

Postepy dermatologii i alergologii
2025

Mycosis Fungoides, Sézary Syndrome, and Cutaneous B-Cell Lymphomas: 2025 Update on Diagnosis, Risk-Stratification, and Management.

American journal of hematology
2025

An evaluation of denileukin diftitox for the treatment of relapsed or refractory cutaneous T-cell lymphoma.

Expert opinion on biological therapy
2025

Racial and Socioeconomic Disparities in Cutaneous T-Cell Lymphoma Survival: Insights From the National Cancer Database.

Clinical lymphoma, myeloma &amp; leukemia
2025

OX40-OX40L Axis in Cutaneous T-Cell Lymphomas: Pathogenic, Prognostic, and Potential Therapeutic Perspectives.

Biomolecules
2025

Mycosis fungoides and IL-4/13 inhibitors: what is known and unmet needs.

Expert review of clinical immunology
2026

Implementing total skin electron irradiation in radiotherapy: a structured change management approach.

Strahlentherapie und Onkologie : Organ der Deutschen Rontgengesellschaft ... [et al]
2025

Dermatoscopic Patterns in Mycosis Fungoides: Observations from a Case-Series Retrospective Analysis and a Review of the Literature.

Diagnostics (Basel, Switzerland)
2025

Case Report: Cutaneous T-cell lymphoma associated with biologic therapy: three cases and a literature review.

Frontiers in medicine
2025

[T follicular helper cell lesions and mimics in dermatopathology: From theory to practice].

Annales de pathologie
2025

Phase II trial of atezolizumab (Anti-PD-L1) in the treatment of relapsed/refractory IIB/IVB mycosis fungoides/Sézary syndrome patients after previous systemic treatment. EORTC-1652-CLTG "PARCT".

European journal of cancer (Oxford, England : 1990)
2025

Concomitant Squamous Cell Carcinoma and Mycosis Fungoides Within the Same Lesion: A Case Report and Literature Review.

The American Journal of dermatopathology
2025

Fatal mycosis fungoides, misdiagnosed as contact dermatitis.

EJNMMI reports
2025

Maturation-Related and Functional-Associated Phenotypic Profile of Tumor T Cells in Mature/Peripheral T-Cell Neoplasms: Association With the Diagnostic Subtype of the Disease.

Laboratory investigation; a journal of technical methods and pathology
2025

Rare Concomitant Langerhans Cell Histiocytosis in a Patient With Folliculotropic Mycosis Fungoides.

Journal of cutaneous pathology
2025

Oral and Topical Janus Kinase Inhibitors in Patients With Cutaneous T-Cell Lymphoma: A Real-World Single-Center Experience.

The Journal of dermatology
2025

Drug- and Vaccine-Induced Cutaneous T-Cell Lymphoma: A Systematic Review of the Literature.

Journal of skin cancer
2025

ASTCT and USCLC clinical practice recommendations for allogeneic stem cell transplant in mycosis fungoides and Sézary syndrome.

Journal of the American Academy of Dermatology
2025

Altered immune cell profiles in blood of mature/peripheral T-cell leukemia/lymphoma patients: an EuroFlow study.

Frontiers in immunology
2025

Pembrolizumab in Relapsed or Refractory Mycosis Fungoides or Sézary Syndrome.

JAMA dermatology
2025

RN7SL1 overexpression promotes cell proliferation in cutaneous T-cell lymphoma via miR-34a-5p/MYCN axis.

Journal of dermatological science
2024

Clinical Utility of TRBC1 Addition in Multiparametric Flow Cytometry for T Cell Lymphoma Diagnosis.

Translational medicine @ UniSa
2025

Sézary Syndrome-Eosinophilia Explained by a Blood Film.

American journal of hematology
2025

Therapeutic advances for cutaneous T-cell lymphoma.

The British journal of dermatology
2025

Central Nervous System Manifestations of Cutaneous Lymphomas.

Current neurology and neuroscience reports
2025

The real-world application of T-cell receptor constant beta-1 chain antibody assay in cutaneous T-cell lymphoma.

British journal of haematology
2025

Increased risk of cutaneous squamous cell carcinomas in patients with Sézary syndrome: A nationwide multicenter retrospective study.

Journal of the American Academy of Dermatology
2025

Identification of subgroups of early-stage mycosis fungoides patients with increased itch and impaired quality of life.

Frontiers in oncology
2025

Systemic Targeted Therapies in Patients with Relapsed/Refractory Advanced Stage Cutaneous T-cell Lymphoma: A Real-world Single-centre Case Series.

Acta dermato-venereologica
2025

Management of Mycosis Fungoides and Sézary Syndrome With Oral Systemic Therapies.

Journal of cutaneous medicine and surgery
2025

Unveiling the Role of the Cellular Tumor Microenvironment and the Therapeutic Targets it Provides in Cutaneous T-Cell Lymphoma.

Current oncology reports
2025

Cutaneous T-cell lymphomas: a real-life experience of anticipated use of mogamulizumab in Italy.

Italian journal of dermatology and venereology
2025

Overall and Cause-Specific Mortality Among Patients With Cutaneous T-Cell Lymphoma in the United States.

EJHaem
2025

Preclinical in vitro and in vivo evidence for targeting CD74 as an effective treatment strategy for cutaneous T-cell lymphomas.

The British journal of dermatology
2024

Diagnostic Approach to Adult Erythroderma: A Rare Case of Sezary Syndrome.

Iranian journal of pathology
2025

Estrogen receptor β stimulation as a possible novel therapeutic target for cutaneous T-cell lymphoma.

Blood advances
2025

Therapeutic Targeting of the Janus Kinase/Signal Transducer and Activator of Transcription Pathway in Cutaneous T-Cell Lymphoma.

Cancers
2025

The CD39/CD73/Adenosine and NAD/CD38/CD203a/CD73 Axis in Cutaneous T-Cell Lymphomas.

Cells
2025

Exploring the Link Between Dupilumab and Cutaneous T-Cell Lymphomas: A Systematic Review.

Dermatitis : contact, atopic, occupational, drug
2024

Sézary syndrome mimicking Steven-Johnson syndrome: A case report.

Medicine
2025

HDAC10 and its implications in Sézary syndrome pathogenesis.

Frontiers in cell and developmental biology
2025

Is dupilumab use in atopic dermatitis associated with cutaneous T-cell lymphoma?

The Journal of allergy and clinical immunology
2025

Mycosis fungoides and Sezary syndrome - Simplifying the approach for dermatologists. Part 2: Evaluation, staging, prognosis and treatment.

Indian journal of dermatology, venereology and leprology
2025

Management of mycosis fungoides and Sézary syndrome with mogamulizumab in combination with psoralen plus UVA: two case reports.

Therapeutic advances in hematology
2025

Insights into treatment of patients with mycosis fungoides or Sézary syndrome using mogamulizumab.

The Journal of dermatological treatment
2024

Sézary syndrome with large cell transformation and T-follicular helper phenotype.

JAAD case reports
2025

Is Kaposi sarcoma a novel comorbidity of cutaneous lymphoma? A systematic review of the literature.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Targeted dual biologic therapy for erythroderma of unknown etiology guided by high-parameter peripheral blood immunophenotyping.

Scientific reports
2025

Treatment of Sezary syndrome with combination romidepsin and tofacitinib: A case report.

JAAD case reports
2025

Advances in Novel Systemic Therapies for the Management of Cutaneous T Cell Lymphoma (CTCL).

Current hematologic malignancy reports
2025

Black patients with Mycosis fungoides and Sézary Syndrome experience worse health-related quality of life: A cross-sectional study.

Journal of the American Academy of Dermatology
2024

Could Residents Adequately Assess the Severity of Skin Lesions in Mycosis Fungoides/Sézary Syndrome? Evaluation of Interrater Agreement and Interrater Reliability of mSWAT.

Journal of clinical medicine
2025

Mycosis fungoides and Sézary syndrome - Simplifying the approach for dermatologists. Part 1: Etiopathogenesis, clinical features and evaluation.

Indian journal of dermatology, venereology and leprology
2025

Practical recommendations for therapy and monitoring of mogamulizumab patients in Germany.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Efficacy and Safety of Denileukin Diftitox-Cxdl, an Improved Purity Formulation of Denileukin Diftitox, in Patients With Relapsed or Refractory Cutaneous T-Cell Lymphoma.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology
2025

A Yale Dermatology perspective on cutaneous T cell lymphoma: Historic reflection to emerging therapies.

Clinics in dermatology
2025

Breaking Down the Barriers for Patients With Cutaneous T-Cell Lymphoma: Current Controversies and Challenges for Radiation Oncologists in 2024.

Seminars in radiation oncology
2025

Differential Expression of p53 in Mycosis Fungoides, Sezary Syndromes, and Their Transformed Forms.

The American Journal of dermatopathology
2024

Immune Checkpoints and Their Inhibition in T-Cell Lymphomas.

Folia biologica
2024

Through thick and thin: confronting the aggressive cutaneous T-cell lymphomas.

Hematology. American Society of Hematology. Education Program
2024

The role of stem cell transplant (auto and allo) in PTCL and CTCL.

Hematology. American Society of Hematology. Education Program
2024

Sustained Complete Remission in Sézary Syndrome using Extracorporeal Photopheresis: A Multicentric Case Series.

Acta dermato-venereologica
2024

Potential mogamulizumab-associated inflammatory bowel disease in cutaneous T-cell lymphoma management.

JAAD case reports
2025

Retrospective cohort study of novel oral agents lenalidomide and duvelisib for relapsed or refractory mycosis fungoides and Sézary syndrome.

Journal of the American Academy of Dermatology
2025

Skin barrier dysfunction in cutaneous T-cell lymphoma: From pathogenic mechanism of barrier damage to treatment.

Critical reviews in oncology/hematology
2024

A Retrospective Comparison Between Home and in-Office NB-UVB Efficacy for Patients With Mycosis Fungoides.

Photodermatology, photoimmunology &amp; photomedicine
2025

Haematogenous seeding in mycosis fungoides and Sézary syndrome: current evidence and clinical implications.

The British journal of dermatology
2024

Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.

EClinicalMedicine
2024

Nanopore Sequencing for T-Cell Receptor Rearrangement Analysis in Cutaneous T-Cell Lymphoma.

Cancers
2025

Cutaneous T-cell lymphoma: Consensus on diagnosis and management in Taiwan.

Journal of the Formosan Medical Association = Taiwan yi zhi
2024

Folliculotropic mycosis fungoides with leukaemic involvement in middle childhood: a rare encounter.

BMJ case reports
2025

Use of Immune Checkpoint Inhibitors in Refractory Mycosis Fungoides and Sézary Syndrome: A Systematic Review.

Journal of cutaneous medicine and surgery
2024

Clinical Reasoning: Progressive Peripheral Neuropathy in a 66-Year-Old Woman With Sezary Syndrome.

Neurology
2024

Radiotherapy in cutaneous lymphomas: Recommendations from the EORTC cutaneous lymphoma tumour group.

European journal of cancer (Oxford, England : 1990)
2024

The Tumor Microenvironment as a Therapeutic Target in Cutaneous T Cell Lymphoma.

Cancers
2024

Role of Retinoids and Their Analogs in the Treatment of Cutaneous T-cell Lymphoma: A Systematic Review.

Cureus
2025

Genetic alteration of class I HLA in cutaneous T-cell lymphoma.

Blood
2024

Real-world Use of Mogamulizumab Among Patients With Mycosis Fungoides and Sézary Syndrome Before and During COVID-19 in the United States.

Clinical therapeutics
2024

Pegylated interferon-α2a in cutaneous T-cell lymphoma - a multicenter retrospective data analysis with 70 patients.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Mogamulizumab-induced bone granuloma.

Joint bone spine
2024

Mogamulizumab and Concomitant Hypofractionated Low-Dose Total Skin Electron Beam Therapy (2 × 4 Gy) in Cutaneous T-Cell Lymphoma: Proof of Principle, Report of Two Cases.

Current oncology (Toronto, Ont.)
2025

Cutaneous T-Cell Lymphoma and Dupilumab Use: A Multifactorial and Complex Story.

The Journal of investigative dermatology
2024

Upadacitinib unmasks cutaneous T-cell lymphoma in atopic dermatitis.

JAAD case reports
2025

Health-related quality of life in cutaneous T-cell lymphoma: A post hoc analysis of a phase 3 trial in mycosis fungoides and Sézary syndrome.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

A Retrospective Cohort Study to Determine Real-World Treatment Patterns in Patients with Sézary Syndrome in the United States.

Oncology and therapy
2024

Systemic treatments with monoclonal antibodies in mycosis fungoides and Sézary syndrome.

Dermatology reports
2024

Durable remission of refractory and advanced stage mycosis fungoides/sezary syndrome utilizing an "outpatient" alemtuzumab, fludarabine-based reduced intensity allogeneic hematopoietic cell transplantation.

Bone marrow transplantation
2024

Evaluation of Sézary cell marker expression and cell death behaviour upon in vitro treatment by flow cytometry in Sézary syndrome patients.

Experimental dermatology
2024

Mogamulizumab for Sézary syndrome: long-term remission with associated autoimmune haemolytic anaemia.

European journal of dermatology : EJD
2024

[Persistent Effects of Mogamulizumab on Peripheral Blood Lesions after Treatment Completion in a Patient with Refractory Sézary Syndrome-A Case Report].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2024

Assessing Health-Related Quality of Life in Mycosis Fungoides and Sézary Syndrome: Unmet Needs.

Cancers
2024

On the Way to Curing Advanced-Stage Mycosis Fungoides/Sézary Syndrome.

Clinical lymphoma, myeloma &amp; leukemia
2024

Quantification of the median fluorescence intensity of CD3 and CD4 in mycosis fungoides/Sezary syndrome versus non-neoplastic control cases in peripheral blood.

Journal of hematopathology
2024

A case report of refractory advanced-stage mycosis fungoides: successful treatment and improved patient quality of life with mogamulizumab.

Therapeutic advances in hematology
2024

The fifth edition of the WHO-Classification - what is new for cutaneous lymphomas?

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Cutaneous T-cell Lymphoma.

Hematology/oncology clinics of North America
2024

Development and psychometric properties of the Functional Assessment of Cancer Therapy-Cutaneous T-Cell Lymphoma (FACT-CTCL) instrument.

The British journal of dermatology
2024

Improving disease-specific survival for patients with Sezary syndrome in the modern era of systemic therapies.

British journal of haematology
2025

JAAD Game Changers: Guidelines for phototherapy of mycosis fungoides and Sézary syndrome: A consensus statement of the US Cutaneous Lymphoma Consortium.

Journal of the American Academy of Dermatology
2024

Progression of CD8-positive Sézary syndrome from mycosis fungoides following dupilumab treatment.

European journal of dermatology : EJD
2024

Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.

EClinicalMedicine
2024

Whole-Transcriptome Sequencing-Based Profiling of the Cutaneous Virome in Patients with Secondary Immunodeficiency.

JID innovations : skin science from molecules to population health
2024

Primary cutaneous EBV+ extranodal NK/T-cell lymphoma of gamma/delta T-cell lineage in the posttransplantation setting.

Journal of cutaneous pathology
2024

A retrospective study of prognostic factors and treatment outcome in advanced-stage Mycosis Fungoides and Sezary Syndrome.

Hematology (Amsterdam, Netherlands)
2024

[Translated article] Survival Analysis and Prognostic Factors in a Case Series of 148 Cutaneous T-Cell Lymphomas.

Actas dermo-sifiliograficas
2024

Mogamulizumab-associated rash - Case series and review of the literature.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Sézary Syndrome in West Sweden: Exploring Epidemiology, Clinical Features, and Treatment Patterns in a Registry-Based Retrospective Analysis.

Cancers
2024

Chart review study of real-world clinical outcomes in patients with cutaneous T-cell lymphoma treated with extracorporeal photopheresis in the US in 2017-2019.

The Journal of dermatological treatment
2024

Updated cutaneous T-cell lymphoma TNMB staging criteria fail to identify patients with Sézary syndrome with low blood burden.

Blood
2024

Fine-Tuning Low-Dose Total Skin Electron Therapy for Optimal Management of Cutaneous T-Cell Lymphoma: A Comparative Analysis of Regimens.

Advances in radiation oncology
Ver todos os 1.318 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Síndrome Sezary.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Síndrome Sezary

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Inducible Costimulator and Its Ligand Promote Proliferation and Migration of Tumor Cells in Cutaneous T-Cell Lymphoma.
    International journal of molecular sciences· 2026· PMID 41683829mais citado
  2. Resminostat for maintenance treatment in patients with advanced-stage mycosis fungoides or S&#xe9;zary syndrome: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.
    The Lancet. Haematology· 2026· PMID 41651641mais citado
  3. Protocol for a mixed-methods modified Delphi study for the development of a core domain set to assess the health-related quality of life of patients with mycosis fungoides and S&#xe9;zary syndrome in clinical trials.
    BMJ open· 2026· PMID 41651511mais citado
  4. T-Cell Receptor Clonotypes and Aggressive Subtypes in Cutaneous T-Cell Lymphoma.
    JAMA dermatology· 2026· PMID 41123901mais citado
  5. Safety of Concurrent Systemic Therapy and Total Skin Electron Therapy in Cutaneous T-Cell Lymphoma.
    Clinical lymphoma, myeloma &amp; leukemia· 2026· PMID 41876351mais citado
  6. Targeting Cutaneous T-cell lymphoma in non-hodgkin lymphoma: What's new for investigational agents?
    Expert Opin Investig Drugs· 2026· PMID 41988788recente
  7. Mogamulizumab-Induced Linear IgA Bullous Dermatosis in a Patient With Sézary Syndrome.
    Australas J Dermatol· 2026· PMID 41980060recente
  8. Dupilumab therapy in atopic dermatitis when cutaneous lymphoma is suspected: Consensus recommendations from the EORTC Cutaneous Lymphoma Tumour Group.
    J Allergy Clin Immunol· 2026· PMID 41967816recente
  9. Ex-vivo Sézary Syndrome patient tumour cells are sensitive to ATR inhibition.
    J Invest Dermatol· 2026· PMID 41962832recente
  10. Conversational Artificial Intelligence-Enabled Molecular Characterization of Sézary Syndrome Reveals Distinct Pathway-Level Alterations Compared with Non-Sézary Cutaneous T-Cell Lymphoma.
    medRxiv· 2026· PMID 41959803recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3162(Orphanet)
  2. MONDO:0017844(MONDO)
  3. GARD:7629(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q2165399(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome Sezary
Compêndio · Raras BR

Síndrome Sezary

ORPHA:3162 · MONDO:0017844
Prevalência
Unknown
Herança
Multigenic/multifactorial, Not applicable
CID-10
C84.1 · Doença de Sézary
CID-11
Ensaios
10 ativos
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0036920
EuropePMC
Wikidata
Wikipedia
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades