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Sezary syndrome

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https://www.readbyqxmd.com/read/27897325/the-biomarker-landscape-in-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#1
REVIEW
Brittany Dulmage, Larisa Geskin, Joan Guitart, Oleg E Akilov
The practice of preemptive individualized medicine is predicated on the discovery, development, and application of biomarkers in specific clinical settings. Mycosis fungoides and Sézary syndrome are the two most common type of cutaneous T-cell lymphoma, yet diagnosis, prognosis, and disease monitoring remain a challenge. In this review, we discuss the current state of biomarker discovery in mycosis fungoides and Sézary syndrome, highlighting the most promising molecules in different compartments. Further, we emphasize the need for continued multicenter efforts to validate available and new biomarkers and to develop prospective combinatorial panels of already discovered molecules...
November 28, 2016: Experimental Dermatology
https://www.readbyqxmd.com/read/27889686/microrna-181-contributes-to-downregulation-of-samhd1-expression-in-cd4-t-cells-derived-from-s%C3%A3-zary-syndrome-patients
#2
Rebecca Kohnken, Karthik M Kodigepalli, Anjali Mishra, Pierluigi Porcu, Li Wu
Sézary syndrome (SS) is a rare subtype of cutaneous T-cell lymphoma (CTCL) that is characterized by aggressive spread of neoplastic CD4+ T-cells from the skin into the bloodstream with metastasis to visceral organs. The deoxynucleoside triphosphohydrolase SAMHD1 is highly expressed in normal CD4+ T-cells, while its expression is down-regulated in CD4+ T-cells from SS patients. MicroRNA (miR) dysregulation is an important epigenetic mechanism in the pathogenesis and progression of SS. MiR-181 has been shown to inhibit SAMHD1 expression in cell lines and was identified as an important prognostic biomarker in CTCL...
November 17, 2016: Leukemia Research
https://www.readbyqxmd.com/read/27873737/erythroderma-and-non-hodgkin-t-cell-lymphoma-what-else-apart-from-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#3
Alessandro Pileri, Cinzia Pellegrini, Claudio Agostinelli, Vieri Grandi, Annalisa Patrizi, Pier Luigi Zinzani, Nicola Pimpinelli
BACKGROUND: Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature. OBJECTIVES: We sought to describe PTCL-NOS cases characterised by erythrodermic dissemination to the skin...
November 21, 2016: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/27828640/alemtuzumab-in-refractory-s%C3%A3-zary-syndrome
#4
Carmen María Alcántara Reifs, Rafael Salido-Vallejo, Gloria María Garnacho-Saucedo, Sofía De la Corte-Sánchez, Alberto González-Menchen, Antonio Vélez García-Nieto
Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27821903/proteomic-approaches-to-biomarker-discovery-in-cutaneous-t-cell-lymphoma
#5
REVIEW
Alexandra Ion, Iris Maria Popa, Laura Maria Lucia Papagheorghe, Cristina Lisievici, Mihai Lupu, Vlad Voiculescu, Constantin Caruntu, Daniel Boda
Cutaneous T-cell lymphoma (CTCL) is the most frequently encountered type of skin lymphoma in humans. CTCL encompasses multiple variants, but the most common types are mycosis fungoides (MF) and Sezary syndrome (SS). While most cases of MF run a mild course over a period of many years, other subtypes of CTCL are very aggressive. The rapidly expanding fields of proteomics and genomics have not only helped increase knowledge concerning the carcinogenesis and tumor biology of CTCL but also led to the discovery of novel markers for targeted therapy...
2016: Disease Markers
https://www.readbyqxmd.com/read/27805921/c-cbl-e3-ubiquitin-ligase-expression-increases-across-the-spectrum-of-benign-and-malignant-t-cell-skin-diseases
#6
Katrin A Salva, Margo J Reeder, Rita Lloyd, Gary S Wood
Prolonged survival of lesional T cells plays a central role in the pathogenesis of T-cell-mediated dermatoses. We have recently shown that the ubiquitin ligase c-CBL is highly expressed in cutaneous T-cell lymphoma (CTCL) and that its knockdown increases activation-induced cell death, a key pathway for T-cell apoptosis. Here, we extend our work on c-CBL expression in malignant T cells to their nonneoplastic counterparts in benign inflammatory dermatoses. Immunohistochemical staining with anti-c-CBL antibody was performed on lesional biopsies from a total of 65 patients with atopic dermatitis, allergic contact dermatitis, pityriasis rosea, psoriasis vulgaris, lichen planus, mycosis fungoides (MF)/Sézary syndrome (SS) as well as on tonsil tissue from 5 individuals and on 5 human CTCL cell lines...
October 31, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27780938/toll-like-receptor-agonists-partially-restore-the-production-of-pro-inflammatory-cytokines-and-type-i-interferon-in-s%C3%A3-zary-syndrome
#7
Kelly C G Manfrere, Marina P Torrealba, Denis R Miyashiro, Luanda M S Oliveira, Gabriel C de Carvalho, Josenilson F Lima, Anna Claudia C C Branco, Nátalli Z Pereira, Juliana Pereira, José A Sanches, Maria N Sato
Sézary syndrome (SS) carries a poor prognosis, and infections represent the most frequent cause of death in SS patients. Toll-like receptors (TLRs) are a family of innate immune receptors that induce protective immune responses against infections. We sought to evaluate the ability of TLR agonists to induce inflammatory cytokine, Th2 cytokine, and type I interferon (IFN-I) production by peripheral blood mononuclear cells (PBMC) of untreated SS patients. We detected impaired IL-6, IL-10 and IL-13 secretion by PBMC induced by the agonists for TLR5, TLR3, TLR7 and TLR9 in SS patients, while it was partially recovered by TLR2/TLR4 and TLR7/8 agonists TNF secretion was restored following stimulation with TLR2/TLR4 agonists...
October 21, 2016: Oncotarget
https://www.readbyqxmd.com/read/27766406/cd164-identifies-cd4-t-cells-highly-expressing-genes-associated-with-malignancy-in-s%C3%A3-zary-syndrome-the-s%C3%A3-zary-signature-genes-fcrl3-tox-and-mir-214
#8
Bernice M Benoit, Neha Jariwala, Geraldine O'Connor, Landon K Oetjen, Timothy M Whelan, Adrienne Werth, Andrea B Troxel, Hélène Sicard, Lisa Zhu, Christopher Miller, Junko Takeshita, Daniel W McVicar, Brian S Kim, Alain H Rook, Maria Wysocka
Sézary syndrome (SS), a leukemic variant of cutaneous T-cell lymphoma (CTCL), is associated with a significantly shorter life expectancy compared to skin-restricted mycosis fungoides. Early diagnosis of SS is, therefore, key to achieving enhanced therapeutic responses. However, the lack of a biomarker(s) highly specific for malignant CD4(+) T cells in SS patients has been a serious obstacle in making an early diagnosis. We recently demonstrated the high expression of CD164 on CD4(+) T cells from Sézary syndrome patients with a wide range of circulating tumor burdens...
October 20, 2016: Archives of Dermatological Research
https://www.readbyqxmd.com/read/27732942/doxycycline-is-an-nf-%C3%AE%C2%BAb-inhibitor-that-induces-apoptotic-cell-death-in-malignant-t-cells
#9
Carolina V Alexander-Savino, Matthew S Hayden, Christopher Richardson, Jiyong Zhao, Brian Poligone
Cutaneous T-cell Lymphoma (CTCL) is a rare non-Hodgkin's lymphoma that can affect the skin, blood, and lymph nodes, and can metastasize at late stages. Novel therapies that target all affected disease compartments and provide longer lasting responses while being safe are needed. One potential therapeutic target is NF-κB, a regulator of immune responses and an important participant in carcinogenesis and cancer progression. As a transcription factor, NF-κB targets genes that promote cell proliferation and survival...
October 6, 2016: Oncotarget
https://www.readbyqxmd.com/read/27704468/s%C3%A3-zary-syndrome-clinical-and-biological-aspects
#10
Rebecca Kohnken, Stephanie Fabbro, Justin Hastings, Pierluigi Porcu, Anjali Mishra
Sézary syndrome (SS) is a rare and aggressive type of cutaneous T cell lymphoma (CTCL) characterized by an intensely pruritic, exfoliative rash, known as erythroderma, with cutaneous and systemic dissemination of clonal CD4+ T cells into the blood and lymph nodes. This review aims to present recent advancements in the biological and clinical aspects of SS. We begin by providing an overview of the diagnostic criteria for SS and reviewing some of its epidemiological and clinical aspects. We then discuss updates in the etiology of this elusive disease and the genetic and molecular landscapes that define it...
October 4, 2016: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/27675686/validation-of-cutaneous-lymphoma-international-prognostic-index-clipi-for-mycosis-fungoides-and-sezary-syndrome
#11
J Jhaveri, H Danish, S Liu, C R Flowers, M J Lechwoicz, K Patel, N Esiashvili, M K Khan
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/27649045/a-retrospective-comparative-outcome-analysis-following-systemic-therapy-in-mycosis-fungoides-and-sezary-syndrome
#12
Walter Hanel, Robert Briski, Charles W Ross, Thomas F Anderson, Mark S Kaminski, Alexandra C Hristov, Ryan A Wilcox
Cutaneous T-cell lymphomas (CTCL), with few exceptions, remain incurable and treatment is largely palliative. We performed a retrospective analysis of systemic treatment outcomes of patients diagnosed with MF/SS. We identified 223 patients with MF/SS evaluated at a single institution from 1997 to 2013. Disease stage at diagnosis, time of treatment, and treatments received were retrospectively analyzed using our CTCL database. The primary endpoint was time to next treatment (TTNT). Treatment outcomes were analyzed using Kaplan-Meier method and comparisons among groups were made using log-rank analysis...
December 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27637355/romidepsin-for-the-treatment-of-relapsed-refractory-cutaneous-t-cell-lymphoma-mycosis-fungoides-s%C3%A3-zary-syndrome-use-in-a-community-setting
#13
REVIEW
Sunil A Reddy
Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of rare non-Hodgkin lymphomas that arise in the skin. In advanced stages, CTCL becomes systemic and is associated with poor prognosis. Diagnosis of CTCL and treatment of early-stage disease with topical therapies often occurs under the care of a dermatologist. Community oncologists see few patients with CTCL due to direct referrals from dermatologists to academic or lymphoma specialty centers. However, some patients will continue to be managed in a community setting...
October 2016: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/27623940/aberrant-microrna-expression-in-tumor-mycosis-fungoides
#14
E Papadavid, M Braoudaki, M Bourdakou, A Lykoudi, V Nikolaou, G Tounta, A Ekonomidi, E Athanasiadis, G Spyrou, C Antoniou, S Kitsiou-Tzeli, D Rigopoulos, A Kolialexi
Herein, miRNA candidates relevant to mycosis fungoides were investigated to provide data on the molecular mechanisms underlying the pathogenesis of the disease. The miRNA expression profile of skin biopsies from patients with tumor stage MF (tMF) and normal donors was compared using miRNA microarrays. Overall, 154 miRNAs were found differentially expressed between tMF and the control cohort with the majority of them being up-regulated (57 %). Among the upregulated miRNAs, miR-3177, miR-514b-3p, miR-1267, and miR-1282 were exclusively detected in 70 % of tMF...
September 13, 2016: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/27613059/an-association-between-newly-diagnosed-cutaneous-t-cell-lymphoma-and-prior-impetigo-a-nested-case-control-study
#15
Ben Boursi, Kevin Haynes, Ronac Mamtani, Yu-Xiao Yang
Colonization with staphylococcus aureus (SA) is associated with disease activity and progression in patients with cutaneous T-cell lymphoma (CTCL) secondary to T-cell activation by bacterial superantigens. The aim of the current study was to evaluate the possible role of SA as an etiologic factor affecting CTCL initiation. We conducted a nested case-control study in a large population-representative database from the UK. Cases were defined as all patients with an incident diagnosis of mycosis fungoides (MF) or Sezary syndrome (SS) between 1995 and 2013...
November 2016: Archives of Dermatological Research
https://www.readbyqxmd.com/read/27592800/tigit-and-helios-are-highly-expressed-on-cd4-t-cells-in-sezary-syndrome-patients
#16
Neha Jariwala, Bernice Benoit, Andrew V Kossenkov, Landon K Oetjen, Timothy M Whelan, Christine M Cornejo, Junko Takeshita, Brian S Kim, Louise C Showe, Maria Wysocka, Alain H Rook
No abstract text is available yet for this article.
September 1, 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27546732/cd8-mycosis-fungoides-clinically-masquerading-as-alopecia-areata
#17
Sapna M Amin, Timothy Tan, Joan Guitart, Maria Colavincenzo, Pedram Gerami, Pedram Yazdan
A 33-year-old female with a 7-year history of CD8-positive hypopigmented mycosis fungoides (MF) involving the trunk and extremities presented with a large well-defined alopecic patch on her frontal scalp. Clinically, this area resembled alopecia areata (AA) and was without hypopigmentation or erythema. A scalp biopsy revealed a non-scarring inflammatory alopecia and a superficial band-like atypical lymphoid infiltrate with prominent epidermotropism. Atypical, predominately CD8-positive lymphocytes were seen surrounding and infiltrating the bulb portion of several hair follicles...
December 2016: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/27542296/global-patterns-of-methylation-in%C3%A2-s%C3%A3-zary-syndrome-provide-insight-into%C3%A2-the%C3%A2-role-of-epigenetics-in%C3%A2-cutaneous%C3%A2-t-cell-lymphoma
#18
Sean Whittaker
van Doorn et al. have defined the DNA methylomes of Sézary cells based on a genome-wide methylation analysis using the Illumina 450K array platform (Illumina, San Diego, CA). Their results show aberrant DNA methylation patterns in CD4-enriched T cells from peripheral blood samples, patterns that are distinct from those of patients with inflammatory erythroderma and from healthy volunteers. Whereas 7.8% of 473,921 5'-cytosine-phosphate-guanine-3' (CpG) sites were hypomethylated, 3.2% showed marked enrichment and selection for hypermethylated CpG sites within the proximal region of gene promoters, including some genes that have previously been shown to be hypermethylated in cutaneous T-cell lymphomas (CTCLs), using standard bisulfite modification techniques...
September 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27540476/an-overview-of-cutaneous-t-cell-lymphomas
#19
REVIEW
Nooshin Bagherani, Bruce R Smoller
Cutaneous T cell lymphomas (CTCLs) are a heterogeneous group of extranodal non-Hodgkin's lymphomas that are characterized by a cutaneous infiltration of malignant monoclonal T lymphocytes. They typically afflict adults with a median age of 55 to 60 years, and the annual incidence is about 0.5 per 100,000. Mycosis fungoides, Sézary syndrome, and primary cutaneous peripheral T cell lymphomas not otherwise specified are the most important subtypes of CTCL. CTCL is a complicated concept in terms of etiopathogenesis, diagnosis, therapy, and prognosis...
2016: F1000Research
https://www.readbyqxmd.com/read/27501224/cutaneous-t-cell-lymphoma-a-hypothesis-on-disease-pathophysiology-involving-deficiency-in-dna-repair
#20
K Thestrup-Pedersen
Cutaneous T-cell lymphoma (CTCL) is a rare disease occurring in Europe among two persons per million per year. It affects men more often than women (2:1). It is primarily a skin disease. In about 20% of patients, it becomes fatal with tumours in the skin and spreading to lymph glands. Approximately 3% of patients show a leukemic form called Sezary's syndrome, where malignant cells are present in blood with accompanying erythrodermia. CTCL is a T-lymphocyte disease occurring late in life as the average age of patients is around 66 years in Europe, Japan and the US...
October 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
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