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Sezary syndrome

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
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
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
Walter Hanel, Robert Briski, Charles W Ross, Thomas F Anderson, Mark S Kaminski, Alexandra C Hristov, Ryan A Wilcox
BACKGROUND: 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 at the University of Michigan. METHODS: We identified 223 patients with MF/SS evaluated at the University of Michigan from 1997-2013. Disease stage at diagnosis, time of treatment, treatments received and reasons for treatment cessation were retrospectively analyzed using our CTCL database...
September 20, 2016: American Journal of Hematology
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
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
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
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
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...
August 22, 2016: Journal of Cutaneous Pathology
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
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
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...
August 8, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Silvia Alberti-Violetti, Pamela Vezzoli, Laura Corti, Daniele Fanoni, Valentina Merlo, Luigia Venegoni, Alberto Reseghetti, Emilio Berti
We describe the case of a 17-year-old Hispanic boy who had had erythroderma and diffuse lymphadenopathy for approximately 6 months. A diagnosis of Sézary syndrome was made on the basis of the histologic features of the skin; the presence of the same T-cell clone on the skin, blood, and bone marrow; and the high CD4(+) lymphocyte count with an aberrant phenotype in peripheral blood; bone marrow involvement was also present. The patient was treated with systemic gemcitabine and achieved partial remission.
September 2016: Pediatric Dermatology
Julia J Scarisbrick
PURPOSE OF REVIEW: Currently, there are no curative therapies for cutaneous T-cell lymphoma (CTCL), and consecutive treatments tend to be given until loss of response. There is an urgent need for new and improved therapies in CTCL to treat symptoms such as pruritus and painful skin lesions and prolong survival. This article reviews new drugs available for CTCL. RECENT FINDINGS: CTCL encompasses a group of T-cell neoplasms presenting in the skin without systemic involvement...
September 2016: Current Opinion in Oncology
Ahmed Haider, Anne Steininger, Reinhard Ullmann, Michael Hummel, Lora Dimitrova, Marc Beyer, Staffan Vandersee, Dido Lenze, Wolfram Sterry, Chalid Assaf, Markus Möbs
The key role of the Runt-related transcription factor 3 (RUNX3) in physiological T-cell differentiation has been extensively documented. However, information on its relevance for the development of human T-cell lymphomas or leukemias is scarce. Here we show that alterations of RUNX3 by either heterozygous deletion or methylation of its distal promoter can be observed in the tumor cells of 15/21 (71%) patients suffering from Sézary syndrome (SS), an aggressive variant of cutaneous T-cell lymphoma. In consequence, mRNA levels of RUNX3/p46, the isoform controlled by the distal promoter, are significantly lower in SS tumor cells...
July 1, 2016: Journal of Investigative Dermatology
Edith Chevret, Jean-Philippe Merlio
Sézary syndrome is probably the most studied cutaneous T-cell lymphoma subtype. Beyond the consensus criteria for Sézary syndrome diagnosis, Sézary cells display heterogeneous phenotypes and differentiation profiles. In the face of SS diversity, the great hope is to develop targeted therapies based on next-generation sequencing to define the genetic landscape of Sézary syndrome. Prasad et al. report on the use of exome sequencing and RNA sequencing to study selected CD4(+) blood cells from 15 patients with erythroderma Sézary syndrome, 14 of whom fulfilled the conventional criteria for diagnosis...
July 2016: Journal of Investigative Dermatology
Henry K Wong
Sézary syndrome can be challenging to differentiate from erythrodermic inflammatory dermatitis. Biomarkers have been identified in Sézary syndrome, but have not been validated in multicenter studies in a cohort that allows comparisons. Boonk et al. now describe results that confirm the value of immunophenotype, and they report higher sensitivity and specificity for a set of genes used to distinguish Sézary syndrome from erythrodermic inflammatory dermatitis.
July 2016: Journal of Investigative Dermatology
Lise M Lindahl, Simon Fredholm, Claudine Joseph, Boye Schnack Nielsen, Lars Jønson, Andreas Willerslev-Olsen, Maria Gluud, Edda Blümel, David L Petersen, Nina Sibbesen, Tengpeng Hu, Claudia Nastasi, Thorbjørn Krejsgaard, Ditte Jæhger, Jenny L Persson, Nigel Mongan, Mariusz A Wasik, Ivan V Litvinov, Denis Sasseville, Sergei B Koralov, Charlotte M Bonefeld, Carsten Geisler, Anders Woetmann, Elisabeth Ralfkiaer, Lars Iversen, Niels Odum
In cutaneous T cell lymphomas (CTCL), miR-21 is aberrantly expressed in skin and peripheral blood and displays anti-apoptotic properties in malignant T cells. It is, however, unclear exactly which cells express miR-21 and what mechanisms regulate miR-21. Here, we demonstrate miR-21 expression in situ in both malignant and reactive lymphocytes as well as stromal cells. qRT-PCR analysis of 47 patients with mycosis fungoides (MF) and Sezary Syndrome (SS) confirmed an increased miR-21 expression that correlated with progressive disease...
June 18, 2016: Oncotarget
Ieva Saulite, Wolfram Hoetzenecker, Stephan Weidinger, Antonio Cozzio, Emmanuella Guenova, Ulrike Wehkamp
Sézary syndrome (SS), an aggressive form of erythrodermic pruritic cutaneous T cell lymphoma (CTCL), from an immunological perspective characterized by increased Th2 cytokine levels, elevated serum IgE and impaired cellular immunity. Not only the clinical appearance but also the hallmark immunological characteristics of SS often share striking similarities with acute flares of atopic dermatitis (AD), a common benign chronic inflammatory skin disease. Given the overlap of several immunological features, the application of similar or even identical therapeutic approaches in certain stages of both diseases may come into consideration...
2016: BioMed Research International
Lucyna Maciejka-Kemblowska, Radoslaw Chaber, Grazyna Wrobel, Jadwiga Maldyk, Marta Kozlowska, Dominika Kulej, Bernarda Kazanowska, Halina Bubala, Bozena Dembowska-Baginska, Grazyna Karolczyk, Andrzej Koltan, Elzbieta Wyrobek
PURPOSE: Peripheral T-cell lymphomas (PTCL) are lymphoproliferative disorders derived from post-thymic cells, that occur extremely rarely in children. The optimal treatment of pediatric PTCL remains still unclear. PATIENTS AND METHODS: Ten children with PTCL from 3 up to 18 years of age registered by the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) were retrospectively analyzed. All patients were treated with different regimens including protocols: for lymphoblastic lymphoma in 7 cases, for anaplastic large cell lymphoma in 1, CHOP in 1...
March 21, 2016: Advances in Medical Sciences
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