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).
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).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 13 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 29 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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.
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
Cell membraneSecreted
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
Cell membraneCell surface
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.
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)
Cell membrane
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.
Variantes genéticas (ClinVar)
191 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 1 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
14 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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
Ensaios em destaque
🟢 Recrutando agora
5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
33 ensaios clínicos encontrados, 10 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.111
Inducible Costimulator and Its Ligand Promote Proliferation and Migration of Tumor Cells in Cutaneous T-Cell Lymphoma.
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.
Resminostat for maintenance treatment in patients with advanced-stage mycosis fungoides or Sézary syndrome: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.
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.
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.
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.
T-Cell Receptor Clonotypes and Aggressive Subtypes in Cutaneous T-Cell Lymphoma.
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.
Safety of Concurrent Systemic Therapy and Total Skin Electron Therapy in Cutaneous T-Cell Lymphoma.
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
Targeting Cutaneous T-cell lymphoma in non-hodgkin lymphoma: What's new for investigational agents?
Mogamulizumab-Induced Linear IgA Bullous Dermatosis in a Patient With Sézary Syndrome.
Dupilumab therapy in atopic dermatitis when cutaneous lymphoma is suspected: Consensus recommendations from the EORTC Cutaneous Lymphoma Tumour Group.
Ex-vivo Sézary Syndrome patient tumour cells are sensitive to ATR inhibition.
Conversational Artificial Intelligence-Enabled Molecular Characterization of Sézary Syndrome Reveals Distinct Pathway-Level Alterations Compared with Non-Sézary Cutaneous T-Cell Lymphoma.
📚 EuropePMC1.318 artigos no totalmostrando 196
Safety of Concurrent Systemic Therapy and Total Skin Electron Therapy in Cutaneous T-Cell Lymphoma.
Clinical lymphoma, myeloma & leukemiaMogamulizumab-associated lymphadenopathy masquerading as lymphoma progression.
JAAD case reportsLeonine Facies in Sézary Syndrome.
Journal of cutaneous medicine and surgeryCutaneous T-cell lymphomas and dupilumab for atopic dermatitis: A systematic review and expert consensus.
Journal of the European Academy of Dermatology and Venereology : JEADVMycosis fungoides/Sézary syndrome and systemic Janus kinase inhibitors: a real-world retrospective study on behalf of the EORTC-CLTG.
The British journal of dermatologyMapping malignant T-cell states and immune circuits in Sézary syndrome by single-cell analysis.
Journal of the European Academy of Dermatology and Venereology : JEADVPersistence of sex differences and stable trends in mortality in patients with mycosis fungoides and Sézary syndrome in Spain (1983-2022).
Medicina clinicaMycosis fungoides and Sézary syndrome.
British journal of haematologyType 2 diabetes and risk of non-Hodgkin lymphoma and multiple myeloma: a pooled analysis.
JNCI cancer spectrumInducible Costimulator and Its Ligand Promote Proliferation and Migration of Tumor Cells in Cutaneous T-Cell Lymphoma.
International journal of molecular sciencesLaboratory Evaluation of Peripheral Blood Involvement in Mycosis Fungoides and Sézary Syndrome: Evolution of Flow Cytometry and Morphology Quantification and Interpretation.
CancersEfficacy and tolerability of mogamulizumab in mycosis fungoides and Sézary Syndrome: a monocentric retrospective study.
Frontiers in oncologyResminostat 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. HaematologyProtocol 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 openA historical review of mycosis fungoides: from Alibert to mogamulizumab.
Skin health and diseaseDiverse transcriptomic and mutational patterns but limited functional pathway alterations in patient-derived Sézary syndrome cells.
The Journal of investigative dermatologyRacial Disparities in United States Clinical Trial Enrollment for Mycosis Fungoides and Sézary Syndrome.
Journal of drugs in dermatology : JDDSézary syndrome arising from cutaneous epitheliotropic T-cell lymphoma, resembling human folliculotropic mycosis fungoides, in a dog.
Journal of comparative pathologyDiagnostic Implications of Dupilumab-Refractory Atopic Dermatitis: Risk of Underlying Cutaneous T-Cell Lymphoma and the Role of Skin Biopsy.
International journal of dermatologyNeutrophil-Galectin-9 Axis Linking Innate and Adaptive Immunity in ATL, Sézary Syndrome, COVID-19, and Psoriasis: An AI-Assisted Integrative Review.
Reports (MDPI)How I manage mycosis fungoides and Sézary syndrome: Current controversies and unmet needs.
British journal of haematologyNovel isochromosome 7q and NOTCH1 mutation in an 88-year-old male with Sézary syndrome.
JAAD case reportsSézary syndrome presenting as vitiligo-like leukoderma with response and repigmentation to mogamulizumab and extracorporeal photopheresis.
JAAD case reportsDelayed Diagnosis of Sézary Syndrome: Lessons From a Psoriasiform Presentation.
Clinical case reportsBrentuximab Vedotin in Advanced-Stage Mycosis Fungoides/Sézary Syndrome with Low CD30 Expression: Real-World Data from the German Cutaneous Lymphoma Network.
CancersIncidence and Mortality of Mycosis Fungoides and Sezary Syndrome: A Nationwide Registry Study in Finland.
Acta dermato-venereologicaMogamulizumab plus etoposide in the management of mycosis fungoides with blood involvement: a case report.
Therapeutic advances in hematologyInitial experience with a novel device for supporting a debilitated patient during total skin electron beam therapy.
Journal of cancer research and therapeutics[18F]FDG PET/CT revealing extensive cutaneous involvement in Sézary syndrome.
Nuclear medicine review. Central & Eastern EuropeCase Report: Dose-dependent response to oclacitinib in a dog with Sézary syndrome.
Frontiers in veterinary scienceTranslation, 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-sifiliograficasRelapsed/refractory mycosis fungoides and Sezary syndrome: biology, clinical manifestations and therapeutic options.
Leukemia & lymphomaClinical Implications of the Skin Microbiota in the Therapy of Cutaneous T Cell Lymphoma: A Scoping Review.
Dermatology and therapyRecent trends of incidence and mortality of cutaneous lymphomas in Germany.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGDisparities in immunotherapy treatment for mycosis fungoides and Sézary syndrome in the National Cancer Database.
Journal of the American Academy of DermatologyUnusual presentation of fungal keratitis and endogenous endophthalmitis in Sézary syndrome: a case report.
American journal of ophthalmology case reportsRetrospective Analysis of Second Primary Malignancies in Patients with Mycosis Fungoides and Sézary Syndrome in Croatia.
Dermatology practical & conceptualOX-40 signaling promotes tumorigenesis in CTCL by regulating ERK activation.
Frontiers in immunologyscRNA-Seq reveals anti-lymphoma immune responses in mogamulizumab-associated skin eruptions.
Journal of the European Academy of Dermatology and Venereology : JEADVIL4/IL13 Inhibition via Dupilumab Reduces Malignant T-cell Proliferation and Promotes Antitumor Immunity in Sézary Syndrome.
Cancer immunology researchThe Evaluation of Skin Infiltration in Mycosis Fungoides/Sézary Syndrome Using the High-Frequency Ultrasonography.
Journal of clinical medicineT-Cell Receptor Clonotypes and Aggressive Subtypes in Cutaneous T-Cell Lymphoma.
JAMA dermatologyA rare case of Sézary syndrome - A diagnostic challenge.
Indian journal of dermatology, venereology and leprologyExtracorporeal photopheresis therapy rapidly changes the cytokine profile and tumor microenvironment in cutaneous T cell lymphoma.
Frontiers in immunologyCo-expression of CD30 and SLFN11 serves as a dual biomarker for the treatment of cutaneous T-cell lymphoma.
NAR cancerLymph node detection of monoclonal T-cell population correlates with poorer prognosis in mycosis fungoides and Sèzary syndrome.
British journal of haematologyDual 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 pathologyAllogeneic hematopoietic cell transplant in cutaneous T-cell lymphomas: recommendations from the EBMT PH&G Committee.
Bone marrow transplantationRacial differences in mycosis fungoides and Sézary syndrome: A multicenter cohort study.
Journal of the American Academy of DermatologyAllogeneic haematopoietic cell transplant in cutaneous T-cell lymphomas: Recommendations from the EBMT PH&G Committee.
Journal of the European Academy of Dermatology and Venereology : JEADVExpanded CD4+CD57+ T-large granular lymphocytes: A diagnostic pitfall in blood staging of mycosis fungoides/Sézary syndrome.
American journal of clinical pathologyA to F Think MF! A memory aid for early recognition of mycosis fungoides/Sézary syndrome.
The British journal of dermatologyWeekly dosing schedule of brentuximab vedotin is well tolerated in mycosis fungoides/Sézary syndrome.
British journal of haematologySézary syndrome as an unusual cause of Loeffler endocarditis.
Revista espanola de cardiologia (English ed.)Mogamulizumab-associated rash and lymphocytopenia coinciding with treatment response of Sézary syndrome.
European journal of dermatology : EJDQuality and duration of responses with mogamulizumab in cutaneous T-cell lymphomas: Insights into long-lasting outcomes.
British journal of haematologyAn Update on Single-Cell RNA Sequencing in Illuminating Disease Mechanisms of Cutaneous T-Cell Lymphoma.
CancersTargeted Radiotherapy in Primary Cutaneous Lymphomas: Precision, Efficacy, and Evolving Strategies.
CancersRecurrence of mogamulizumab-associated rash in patients with relapsed erythrodermic cutaneous T-cell lymphoma after retreatment with mogamulizumab.
JAAD case reportsSkin Response to Mogamulizumab in Patients With Mycosis Fungoides and Sezary Syndrome: A Retrospective, Real World Cohort.
Clinical lymphoma, myeloma & leukemiaEconomic burden and health-related quality of life in Chinese patients with mycosis fungoides and Sézary syndrome.
Cancer pathogenesis and therapyExpressed mutated genes in Sezary syndrome and their potential prognostic value in patients treated with extracorporeal photopheresis.
Frontiers in immunologySynergistic Efficacy of Chidamide and LB100 in Sézary Syndrome via TNC Downregulation and PI3K/AKT/mTOR Dephosphorylation.
Cancer scienceHigh-Throughput Sequencing and Estimated Relapse Risk in Patients With Sézary Syndrome.
JAMA dermatologyJanus kinase inhibitors - a role for the treatment of cutaneous T-cell lymphomas?
Oncology reviewsUnraveling the role of chemokines in cutaneous T-cell lymphoma: expression levels at different stages.
Frontiers in immunologyClinical 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 HaematologyLow-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 : JDDGCase report of non-gene editing CD7 CAR T cell therapy in CD7+ Sézary syndrome: preclinical validation and first-in-human use.
Frontiers in immunologyCRISPR-Cas9 screen reveals that inhibition of enhancer of zeste homolog 2 sensitizes malignant T cells to dimethyl-fumarate-induced cell death.
The FEBS journalPruritus Is an Indicator for Quality of Life in Cutaneous T-Cell Lymphoma.
The Journal of dermatologyPsychosocial burden of patients with mycosis fungoides and Sézary syndrome compared to other dermatological patients. Pilot study.
Postepy dermatologii i alergologiiA new prognostic index (CLIPI) for advanced cutaneous lymphoma enables precise patient risk stratification.
BloodImmune pathways, current and potential therapies in Mycosis fungoides and Sezary syndrome.
Expert review of clinical immunologyPharmacovigilance study for the identification of mogamulizumab-induced immune-related adverse events using a real-world database.
The oncologistNew nonchemotherapy treatment options for cutaneous T-cell lymphomas an update.
Expert review of anticancer therapyAcneiform type of mogamulizumab-associated rash.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGLinperlisib Plus Chidamide in Relapsed or Refractory Cutaneous T-Cell Lymphoma: A Nonrandomized Clinical Trial.
JAMA dermatologyCoexistence 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 : IMAJIncreased 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 dermatologyHigh Prevalence and Risk Factors for Infection with Human T-Lymphotropic Viruses 1 and 2 in the Municipality of Ananindeua, Pará, Northern Brazil.
VirusesCCR8 Immunohistochemistry Highlights CCR8 as a Diagnostic and Therapeutic Biomarker in Cutaneous T-Cell Lymphoma.
The Journal of investigative dermatologyStructural 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 sciencesLarge-cell transformation of mycosis fungoides: Patterns of care and patient outcomes.
British journal of haematologyEvaluating mogamulizumab in the treatment of primary cutaneous T-cell lymphoma.
ImmunotherapyA phase 1 study of interleukin-15 in combination with mogamulizumab in relapsed and refractory T-cell malignancies.
Blood neoplasiaClassical and biological treatments in mycosis fungoides/Sézary syndrome. New horizons in oncodermatology.
Postepy dermatologii i alergologiiMycosis Fungoides, Sézary Syndrome, and Cutaneous B-Cell Lymphomas: 2025 Update on Diagnosis, Risk-Stratification, and Management.
American journal of hematologyAn evaluation of denileukin diftitox for the treatment of relapsed or refractory cutaneous T-cell lymphoma.
Expert opinion on biological therapyRacial and Socioeconomic Disparities in Cutaneous T-Cell Lymphoma Survival: Insights From the National Cancer Database.
Clinical lymphoma, myeloma & leukemiaOX40-OX40L Axis in Cutaneous T-Cell Lymphomas: Pathogenic, Prognostic, and Potential Therapeutic Perspectives.
BiomoleculesMycosis fungoides and IL-4/13 inhibitors: what is known and unmet needs.
Expert review of clinical immunologyImplementing total skin electron irradiation in radiotherapy: a structured change management approach.
Strahlentherapie und Onkologie : Organ der Deutschen Rontgengesellschaft ... [et al]Dermatoscopic Patterns in Mycosis Fungoides: Observations from a Case-Series Retrospective Analysis and a Review of the Literature.
Diagnostics (Basel, Switzerland)Case Report: Cutaneous T-cell lymphoma associated with biologic therapy: three cases and a literature review.
Frontiers in medicine[T follicular helper cell lesions and mimics in dermatopathology: From theory to practice].
Annales de pathologiePhase 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)Concomitant Squamous Cell Carcinoma and Mycosis Fungoides Within the Same Lesion: A Case Report and Literature Review.
The American Journal of dermatopathologyFatal mycosis fungoides, misdiagnosed as contact dermatitis.
EJNMMI reportsMaturation-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 pathologyRare Concomitant Langerhans Cell Histiocytosis in a Patient With Folliculotropic Mycosis Fungoides.
Journal of cutaneous pathologyOral and Topical Janus Kinase Inhibitors in Patients With Cutaneous T-Cell Lymphoma: A Real-World Single-Center Experience.
The Journal of dermatologyDrug- and Vaccine-Induced Cutaneous T-Cell Lymphoma: A Systematic Review of the Literature.
Journal of skin cancerASTCT and USCLC clinical practice recommendations for allogeneic stem cell transplant in mycosis fungoides and Sézary syndrome.
Journal of the American Academy of DermatologyAltered immune cell profiles in blood of mature/peripheral T-cell leukemia/lymphoma patients: an EuroFlow study.
Frontiers in immunologyPembrolizumab in Relapsed or Refractory Mycosis Fungoides or Sézary Syndrome.
JAMA dermatologyRN7SL1 overexpression promotes cell proliferation in cutaneous T-cell lymphoma via miR-34a-5p/MYCN axis.
Journal of dermatological scienceClinical Utility of TRBC1 Addition in Multiparametric Flow Cytometry for T Cell Lymphoma Diagnosis.
Translational medicine @ UniSaSézary Syndrome-Eosinophilia Explained by a Blood Film.
American journal of hematologyTherapeutic advances for cutaneous T-cell lymphoma.
The British journal of dermatologyCentral Nervous System Manifestations of Cutaneous Lymphomas.
Current neurology and neuroscience reportsThe real-world application of T-cell receptor constant beta-1 chain antibody assay in cutaneous T-cell lymphoma.
British journal of haematologyIncreased risk of cutaneous squamous cell carcinomas in patients with Sézary syndrome: A nationwide multicenter retrospective study.
Journal of the American Academy of DermatologyIdentification of subgroups of early-stage mycosis fungoides patients with increased itch and impaired quality of life.
Frontiers in oncologySystemic Targeted Therapies in Patients with Relapsed/Refractory Advanced Stage Cutaneous T-cell Lymphoma: A Real-world Single-centre Case Series.
Acta dermato-venereologicaManagement of Mycosis Fungoides and Sézary Syndrome With Oral Systemic Therapies.
Journal of cutaneous medicine and surgeryUnveiling the Role of the Cellular Tumor Microenvironment and the Therapeutic Targets it Provides in Cutaneous T-Cell Lymphoma.
Current oncology reportsCutaneous T-cell lymphomas: a real-life experience of anticipated use of mogamulizumab in Italy.
Italian journal of dermatology and venereologyOverall and Cause-Specific Mortality Among Patients With Cutaneous T-Cell Lymphoma in the United States.
EJHaemPreclinical in vitro and in vivo evidence for targeting CD74 as an effective treatment strategy for cutaneous T-cell lymphomas.
The British journal of dermatologyDiagnostic Approach to Adult Erythroderma: A Rare Case of Sezary Syndrome.
Iranian journal of pathologyEstrogen receptor β stimulation as a possible novel therapeutic target for cutaneous T-cell lymphoma.
Blood advancesTherapeutic Targeting of the Janus Kinase/Signal Transducer and Activator of Transcription Pathway in Cutaneous T-Cell Lymphoma.
CancersThe CD39/CD73/Adenosine and NAD/CD38/CD203a/CD73 Axis in Cutaneous T-Cell Lymphomas.
CellsExploring the Link Between Dupilumab and Cutaneous T-Cell Lymphomas: A Systematic Review.
Dermatitis : contact, atopic, occupational, drugSézary syndrome mimicking Steven-Johnson syndrome: A case report.
MedicineHDAC10 and its implications in Sézary syndrome pathogenesis.
Frontiers in cell and developmental biologyIs dupilumab use in atopic dermatitis associated with cutaneous T-cell lymphoma?
The Journal of allergy and clinical immunologyMycosis fungoides and Sezary syndrome - Simplifying the approach for dermatologists. Part 2: Evaluation, staging, prognosis and treatment.
Indian journal of dermatology, venereology and leprologyManagement of mycosis fungoides and Sézary syndrome with mogamulizumab in combination with psoralen plus UVA: two case reports.
Therapeutic advances in hematologyInsights into treatment of patients with mycosis fungoides or Sézary syndrome using mogamulizumab.
The Journal of dermatological treatmentSézary syndrome with large cell transformation and T-follicular helper phenotype.
JAAD case reportsIs 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 : JDDGTargeted dual biologic therapy for erythroderma of unknown etiology guided by high-parameter peripheral blood immunophenotyping.
Scientific reportsTreatment of Sezary syndrome with combination romidepsin and tofacitinib: A case report.
JAAD case reportsAdvances in Novel Systemic Therapies for the Management of Cutaneous T Cell Lymphoma (CTCL).
Current hematologic malignancy reportsBlack patients with Mycosis fungoides and Sézary Syndrome experience worse health-related quality of life: A cross-sectional study.
Journal of the American Academy of DermatologyCould 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 medicineMycosis fungoides and Sézary syndrome - Simplifying the approach for dermatologists. Part 1: Etiopathogenesis, clinical features and evaluation.
Indian journal of dermatology, venereology and leprologyPractical recommendations for therapy and monitoring of mogamulizumab patients in Germany.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGEfficacy 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 OncologyA Yale Dermatology perspective on cutaneous T cell lymphoma: Historic reflection to emerging therapies.
Clinics in dermatologyBreaking Down the Barriers for Patients With Cutaneous T-Cell Lymphoma: Current Controversies and Challenges for Radiation Oncologists in 2024.
Seminars in radiation oncologyDifferential Expression of p53 in Mycosis Fungoides, Sezary Syndromes, and Their Transformed Forms.
The American Journal of dermatopathologyImmune Checkpoints and Their Inhibition in T-Cell Lymphomas.
Folia biologicaThrough thick and thin: confronting the aggressive cutaneous T-cell lymphomas.
Hematology. American Society of Hematology. Education ProgramThe role of stem cell transplant (auto and allo) in PTCL and CTCL.
Hematology. American Society of Hematology. Education ProgramSustained Complete Remission in Sézary Syndrome using Extracorporeal Photopheresis: A Multicentric Case Series.
Acta dermato-venereologicaPotential mogamulizumab-associated inflammatory bowel disease in cutaneous T-cell lymphoma management.
JAAD case reportsRetrospective cohort study of novel oral agents lenalidomide and duvelisib for relapsed or refractory mycosis fungoides and Sézary syndrome.
Journal of the American Academy of DermatologySkin barrier dysfunction in cutaneous T-cell lymphoma: From pathogenic mechanism of barrier damage to treatment.
Critical reviews in oncology/hematologyA Retrospective Comparison Between Home and in-Office NB-UVB Efficacy for Patients With Mycosis Fungoides.
Photodermatology, photoimmunology & photomedicineHaematogenous seeding in mycosis fungoides and Sézary syndrome: current evidence and clinical implications.
The British journal of dermatologyReal-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.
EClinicalMedicineNanopore Sequencing for T-Cell Receptor Rearrangement Analysis in Cutaneous T-Cell Lymphoma.
CancersCutaneous T-cell lymphoma: Consensus on diagnosis and management in Taiwan.
Journal of the Formosan Medical Association = Taiwan yi zhiFolliculotropic mycosis fungoides with leukaemic involvement in middle childhood: a rare encounter.
BMJ case reportsUse of Immune Checkpoint Inhibitors in Refractory Mycosis Fungoides and Sézary Syndrome: A Systematic Review.
Journal of cutaneous medicine and surgeryClinical Reasoning: Progressive Peripheral Neuropathy in a 66-Year-Old Woman With Sezary Syndrome.
NeurologyRadiotherapy in cutaneous lymphomas: Recommendations from the EORTC cutaneous lymphoma tumour group.
European journal of cancer (Oxford, England : 1990)The Tumor Microenvironment as a Therapeutic Target in Cutaneous T Cell Lymphoma.
CancersRole of Retinoids and Their Analogs in the Treatment of Cutaneous T-cell Lymphoma: A Systematic Review.
CureusGenetic alteration of class I HLA in cutaneous T-cell lymphoma.
BloodReal-world Use of Mogamulizumab Among Patients With Mycosis Fungoides and Sézary Syndrome Before and During COVID-19 in the United States.
Clinical therapeuticsPegylated 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 : JDDGMogamulizumab-induced bone granuloma.
Joint bone spineMogamulizumab 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.)Cutaneous T-Cell Lymphoma and Dupilumab Use: A Multifactorial and Complex Story.
The Journal of investigative dermatologyUpadacitinib unmasks cutaneous T-cell lymphoma in atopic dermatitis.
JAAD case reportsHealth-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 : JEADVA Retrospective Cohort Study to Determine Real-World Treatment Patterns in Patients with Sézary Syndrome in the United States.
Oncology and therapySystemic treatments with monoclonal antibodies in mycosis fungoides and Sézary syndrome.
Dermatology reportsDurable remission of refractory and advanced stage mycosis fungoides/sezary syndrome utilizing an "outpatient" alemtuzumab, fludarabine-based reduced intensity allogeneic hematopoietic cell transplantation.
Bone marrow transplantationEvaluation of Sézary cell marker expression and cell death behaviour upon in vitro treatment by flow cytometry in Sézary syndrome patients.
Experimental dermatologyMogamulizumab for Sézary syndrome: long-term remission with associated autoimmune haemolytic anaemia.
European journal of dermatology : EJD[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 & chemotherapyAssessing Health-Related Quality of Life in Mycosis Fungoides and Sézary Syndrome: Unmet Needs.
CancersOn the Way to Curing Advanced-Stage Mycosis Fungoides/Sézary Syndrome.
Clinical lymphoma, myeloma & leukemiaQuantification of the median fluorescence intensity of CD3 and CD4 in mycosis fungoides/Sezary syndrome versus non-neoplastic control cases in peripheral blood.
Journal of hematopathologyA case report of refractory advanced-stage mycosis fungoides: successful treatment and improved patient quality of life with mogamulizumab.
Therapeutic advances in hematologyThe fifth edition of the WHO-Classification - what is new for cutaneous lymphomas?
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGCutaneous T-cell Lymphoma.
Hematology/oncology clinics of North AmericaDevelopment and psychometric properties of the Functional Assessment of Cancer Therapy-Cutaneous T-Cell Lymphoma (FACT-CTCL) instrument.
The British journal of dermatologyImproving disease-specific survival for patients with Sezary syndrome in the modern era of systemic therapies.
British journal of haematologyJAAD 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 DermatologyProgression of CD8-positive Sézary syndrome from mycosis fungoides following dupilumab treatment.
European journal of dermatology : EJDReal-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.
EClinicalMedicineWhole-Transcriptome Sequencing-Based Profiling of the Cutaneous Virome in Patients with Secondary Immunodeficiency.
JID innovations : skin science from molecules to population healthPrimary cutaneous EBV+ extranodal NK/T-cell lymphoma of gamma/delta T-cell lineage in the posttransplantation setting.
Journal of cutaneous pathologyA retrospective study of prognostic factors and treatment outcome in advanced-stage Mycosis Fungoides and Sezary Syndrome.
Hematology (Amsterdam, Netherlands)[Translated article] Survival Analysis and Prognostic Factors in a Case Series of 148 Cutaneous T-Cell Lymphomas.
Actas dermo-sifiliograficasMogamulizumab-associated rash - Case series and review of the literature.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGSézary Syndrome in West Sweden: Exploring Epidemiology, Clinical Features, and Treatment Patterns in a Registry-Based Retrospective Analysis.
CancersChart 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 treatmentUpdated cutaneous T-cell lymphoma TNMB staging criteria fail to identify patients with Sézary syndrome with low blood burden.
BloodFine-Tuning Low-Dose Total Skin Electron Therapy for Optimal Management of Cutaneous T-Cell Lymphoma: A Comparative Analysis of Regimens.
Advances in radiation oncologyAssociações
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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.
- Inducible Costimulator and Its Ligand Promote Proliferation and Migration of Tumor Cells in Cutaneous T-Cell Lymphoma.
- Resminostat for maintenance treatment in patients with advanced-stage mycosis fungoides or Sézary syndrome: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.
- 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.
- T-Cell Receptor Clonotypes and Aggressive Subtypes in Cutaneous T-Cell Lymphoma.
- Safety of Concurrent Systemic Therapy and Total Skin Electron Therapy in Cutaneous T-Cell Lymphoma.
- Targeting Cutaneous T-cell lymphoma in non-hodgkin lymphoma: What's new for investigational agents?
- Mogamulizumab-Induced Linear IgA Bullous Dermatosis in a Patient With Sézary Syndrome.
- Dupilumab therapy in atopic dermatitis when cutaneous lymphoma is suspected: Consensus recommendations from the EORTC Cutaneous Lymphoma Tumour Group.
- Ex-vivo Sézary Syndrome patient tumour cells are sensitive to ATR inhibition.
- Conversational Artificial Intelligence-Enabled Molecular Characterization of Sézary Syndrome Reveals Distinct Pathway-Level Alterations Compared with Non-Sézary Cutaneous T-Cell Lymphoma.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:3162(Orphanet)
- MONDO:0017844(MONDO)
- GARD:7629(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- 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
