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Sezary syndrome

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https://www.readbyqxmd.com/read/29334836/snf5-deficiency-induces-apoptosis-resistance-by-repressing-satb1-expression-in-s%C3%A3-zary-syndrome
#1
Yang Li, Jin Wang, Minghang Yu, Yang Wang, Huilai Zhang, Jie Yin, Zexing Li, Ting Li, Han Yan, Fajin Li, Xi Wang
SNF5, is a core member of the SWI/SNF chromatin remodeling complex. It's deficiency leads to multiple types of aggressive cancer. Sézary syndrome, a leukemic variant of cutaneous T-cell lymphoma, is characterized by its resistance to apoptosis. Although the cause of apoptosis resistance is still poorly understood, recent evidence has revealed the importance of SATB1 in the apoptosis resistance of Sézary syndrome. In this study, we show that SNF5 is an upstream regulator of SATB1 in several conditions and that both are deficient in Sézary cells...
January 16, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29327377/s%C3%A3-zary-syndrome-managed-with-histone-deacetylase-inhibitor-followed-by-anti-ccr4-monoclonal-antibody
#2
T Numata, T Nagatani, K Shirai, T Maeda, K Mae, M Nakasu, M Saito, T Usuda, R Tsuboi, Y Okubo
A 70-year-old man presented to our clinic with a 10-year history of recurrent pruritic erythema and plaques on his trunk and limbs. Based on the pathological findings and monoclonal rearrangement of the T-cell receptor (TCR)-Cβ1 gene, mycosis fungoides (T2N0M0B0 stage IB) was diagnosed. Despite combination therapy including histone deacetylase inhibitor (vorinostat), the symptoms slowly evolved into Sézary syndrome (SS; T4N1M0B2) over 4 years, with dense infiltrates due to atypical lymphocytes expressing CCR4 developing in the entire dermis...
January 12, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29314201/increased-interleukin-36%C3%AE-expression-in-skin-and-sera-of-patients-with-atopic-dermatitis-and-mycosis-fungoides-s%C3%A3-zary-syndrome
#3
Sayaka Otobe, Makoto Sugaya, Rina Nakajima, Tomonori Oka, Naomi Takahashi, Miyoko Kabasawa, Tomomitsu Miyagaki, Yoshihide Asano, Shinichi Sato
Interleukin (IL)-36γ is expressed by keratinocytes and functions as a key initiator of inflammation in the skin. IL-36γ expression is enhanced by tumor necrosis factor-α and IL-17A, having a strong association with psoriasis. In this study, we examined the role of IL-36γ in atopic dermatitis (AD) and mycosis fungoides (MF)/Sézary syndrome (SS). Serum levels of IL-36γ in AD patients and MF/SS patients were elevated compared with those of healthy controls. Importantly, serum IL-36γ levels in AD patients positively correlated with Eczema Area and Severity Index and those of MF/SS patients positively correlated with serum soluble IL-2 receptor levels...
January 3, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29238954/il-25-is-involved-in-ctcl-progression-by-establishing-th2-dominant-microenvironment
#4
R Nakajima, T Miyagaki, T Oka, N Takahashi, M Hirakawa, H Suga, A Yoshizaki, H Fujita, Y Asano, M Sugaya, S Sato
BACKGROUND: Interleukin (IL)-25 is a member of the IL-17 family which can promote and augment T-helper type (Th) 2 responses. The expression of IL-25 and its cognate receptor, IL-25 receptor (IL-25R), is upregulated and correlated with disease activity in Th2-associated diseases. OBJECTIVE: To examine the expression and function of IL-25 in cutaneous T-cell lymphoma (CTCL). METHODS: Expression and localization of IL-25 in lesional skin was investigated using immunohistochemistry...
December 13, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/29221181/analysis-of-ctcl-cell-lines-reveals-important-differences-between-mycosis-fungoides-s%C3%A3-zary-syndrome-vs-htlv-1-leukemic-cell-lines
#5
Elena Netchiporouk, Jennifer Gantchev, Matthew Tsang, Philippe Thibault, Andrew K Watters, John-Douglas Matthew Hughes, Feras M Ghazawi, Anders Woetmann, Niels Ødum, Denis Sasseville, Ivan V Litvinov
HTLV-1 is estimated to affect ~20 million people worldwide and in ~5% of carriers it produces Adult T-Cell Leukemia/Lymphoma (ATLL), which can often masquerade and present with classic erythematous pruritic patches and plaques that are typically seen in Mycosis Fungoides (MF) and Sézary Syndrome (SS), the most recognized variants of Cutaneous T-Cell Lymphomas (CTCL). For many years the role of HTLV-1 in the pathogenesis of MF/SS has been hotly debated. In this study we analyzed CTCL vs. HTLV-1+ leukemic cells...
November 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29205518/therapeutic-and-prognostic-significance-of-parp-1-in-advanced-mycosis-fungoides-and-sezary-syndrome
#6
David Lemchak, Swati Banerjee, Shaunak S Digambar, Brian L Hood, Thomas P Conrads, Jaroslav Jedrych, Larisa Geskin, Oleg E Akilov
While mycosis fungoides (MF) is typically an indolent malignancy, it may infrequently undertake an aggressive course. We used proteomic analyses to identify a biomarker of the aggressive course of MF. Results of this investigation demonstrated that PARP-1, heat shock protein family A (Hsp70) member 1 like (HSAP1L), Hsp70 member 1A (HSPA1A), ATP-depending RNA-helicase (DDX17), and the α isoform of lamina-associated polypeptide 2 (TMPO) had higher expression in aggressive disease versus non-aggressive. Moreover, PARP-1 was overexpressed in patients with early stage of MF who developed later an aggressive disease...
December 5, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/29204699/therapeutic-reduction-of-cell-mediated-immunosuppression-in-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#7
Larisa J Geskin, Oleg E Akilov, Soonyou Kwon, Michael Schowalter, Simon Watkins, Theresa L Whiteside, Lisa H Butterfield, Louis D Falo
Tumor progression is associated with progressive immunosuppression mediated in part by T regulatory cell(s) (Treg) and/or myeloid-derived suppressor cell(s) (MDSC). Development of strategies to reduce populations of immune cells with suppressive function in cancer patients may enable the induction or recovery of immunity against tumor cells, which may limit or reverse disease progression. With a goal of developing Treg and MDSC neutralizing strategies to treat mycosis fungoides (MF) and Sézary syndrome (SzS), we determined the association between disease stage and suppressor cell populations in patients with MF/SzS, including those responding to therapy...
December 4, 2017: Cancer Immunology, Immunotherapy: CII
https://www.readbyqxmd.com/read/29190907/profile-of-differentially-expressed-toll-like-receptor-signaling-genes-in-the-natural-killer-cells-of-patients-with-s%C3%A3-zary-syndrome
#8
Kelly C G ManfrereC, Marina P Torrealba, Denis R Miyashiro, Nátalli Z Pereira, Fabio S Y Yoshikawa, Luana de M Oliveira, Jade Cury-Martins, Alberto J S Duarte, José A Sanches, Maria N Sato
Sézary syndrome (SS), an aggressive and leukemic form of cutaneous T-cell lymphoma, usually results in shortened survival. Improving innate immunity in SS by targeting natural killer (NK) cells with Toll-like receptor (TLR) agonists could be an interesting modulatory strategy. We evaluated the NK cell populations in SS patients assessing activating and inhibitory receptors expression and profiled the differential expression of TLR signaling pathway genes in unstimulated NK cells and after TLR7/8 stimulation...
November 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29187849/quantification-of-inter-sample-differences-in-t-cell-receptor-repertoires-using-sequence-based-information
#9
Ryo Yokota, Yuki Kaminaga, Tetsuya J Kobayashi
Inter-sample comparisons of T-cell receptor (TCR) repertoires are crucial for gaining a better understanding of the immunological states determined by different collections of T cells from different donor sites, cell types, and genetic and pathological backgrounds. For quantitative comparison, most previous studies utilized conventional methods in ecology, which focus on TCR sequences that overlap between pairwise samples. Some recent studies attempted another approach that is categorized into Poisson abundance models using the abundance distribution of observed TCR sequences...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29171395/incidence-and-ten-year-follow-up-of-primary-cutaneous-lymphomas-a-single-centre-cohort-study
#10
Martina Maurelli, Gianpaolo Tessari, Chiara Colato, Donatella Schena, Giampiero Girolomoni
BACKGROUND: Primary cutaneous lymphomas (PCLs) are a rare group of extranodal non-Hodgkin lymphomas, and epidemiological data in Mediterranean countries are scarce. OBJECTIVE: To investigate the incidence and characteristics of PCL in a single tertiary referral centre in Italy. MATERIALS & METHODS: A total of 141 PCL patients, seen over a 10-year follow-up period, were investigated. RESULTS: Incidence rate of PCL was 0...
November 24, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29169156/folliculotropism-does-not-affect-overall-survival-in-mycosis-fungoides-results-from-a-single-center-cohort-and-meta-analysis
#11
Mariah Giberson, Ahmed Mourad, Robert Gniadecki
BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a distinct subtype of mycosis fungoides (MF) with unique clinicopathological features. The medical literature suggests that FMF has a more aggressive course and worse survival than classic MF. Previous studies do not use standardized treatment, and no studies have reported an association between treatment response and overall survival (OS). OBJECTIVE: To compare OS for MF, FMF, and Sézary syndrome (SS) patients...
November 24, 2017: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/29166501/primary-cutaneous-t-cell-lymphoma-experience-from-the-peruvian-national-cancer-institute
#12
Rosana Ruiz, Zaida Morante, Raul Mantilla, Luis Mas, Luis Casanova, Henry L Gomez
BACKGROUND: Primary cutaneous T-cell lymphomas constitute a heterogeneous and rare group of diseases with regional particularities in Latin America. OBJECTIVE: To determine the clinicopathological features, relative frequency and survival among patients from a Peruvian institution. METHODS: Primary cutaneous T-cell lymphomas were defined based on the absence of extracutaneous disease at diagnosis. Classification was performed following the 2008 World Health Organization Classification of Neoplasms of the Hematopoietic and Lymphoid tissues...
September 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29107664/eruption-of-lymphocyte-recovery-with-atypical-lymphocytes-mimicking-a-primary-cutaneous-t-cell-lymphoma-a-series-of-12-patients
#13
Charlotte Hurabielle, Emilie Sbidian, Helmut Beltraminelli, Brigitte Bouchindhomme, Catherine Chassagne-Clément, Brigitte Balme, Céline Bossard, Marie-Hélène Delfau-Larue, Pierre Wolkenstein, Olivier Chosidow, Catherine Cordonnier, Andrea Toma, Cécile Pautas, Nicolas Ortonne
Eruption of lymphocyte recovery (ELR) may occur during bone marrow aplasia after chemotherapies. We reviewed the clinical and pathologic features of 12 patients (male/female: 7/5, median age: 61years) with an atypical ELR histologically mimicking a primary cutaneous T-cell lymphoma (CTCL) such as Sézary Syndrome or CD30+ T-cell lymphoproliferative disorder (LPD). All the patients displayed an erythematous maculopapular eruption on the trunk and the limbs, associated with fever. All but one had received a polychemotherapy for an acute myeloid leukemia (n=10) or an urothelial carcinoma (n=1) before the occurrence of the skin eruption...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29068084/regional-incidences-of-adult-t-cell-leukemia-lymphoma-with-cutaneous-involvement-in-japan
#14
Toshihisa Hamada, Hayato Nomura, Keiji Iwatsuki
Between 2008 and 2015, 462 newly-diagnosed adult T-cell leukemia/lymphoma (ATLL) patients with cutaneous involvement were found from the nationwide registry for Japanese patients with cutaneous lymphoma, of which 391 were selected for the study. They ranged in age from 28 to 93 years (median, 69 years), and included 215 men and 176 women (male : female ratio = 1.2). The 391 patients comprised 193 (50%) with smoldering type, 52 (13%) with chronic type, 44 (11%) with lymphoma type and 102 (26%) with acute type...
October 25, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/29057261/aprepitant-for-the-treatment-of-chronic-refractory-pruritus
#15
REVIEW
Alice He, Jihad M Alhariri, Ronald J Sweren, Madan M Kwatra, Shawn G Kwatra
Chronic pruritus is a difficult condition to treat and is associated with several comorbidities, including insomnia, depression, and decreased quality of life. Treatment for chronic itch includes corticosteroids, antihistamines, and systemic therapies such as naltrexone, gabapentin, UV light therapy, and immunomodulatory treatments, including azathioprine, methotrexate, and cellcept. However, some patients still remain refractory to conventional therapy. Aprepitant is a neurokinin-1 receptor antagonist approved for the prevention of chemotherapy-induced and postoperative nausea and vomiting (CINV, PONV)...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29036928/plasma-mir-155-mir-203-and-mir-205-are-biomarkers-for-monitoring-of-primary-cutaneous-t-cell-lymphomas
#16
Nina Dusílková, Petra Bašová, Jindřich Polívka, Ondřej Kodet, Vojtěch Kulvait, Michal Pešta, Marek Trněný, Tomáš Stopka
Primary cutaneous T-cell lymphomas (CTCL) affect the skin and tend to transform and spread. CTCL involves primarily the Mycosis fungoides (MF) and more aggressive Sezary syndrome (SS). Oncogenic microRNAs (miRs) are stable epigenetic inhibitors often deregulated in the tumour and detectable as biomarkers in non-cellular fractions of peripheral blood. The tumour-specific expression of miR-155, miR-203, and miR-205 was shown to correctly diagnose CTCL. We herein asked whether these microRNAs can be used as plasma biomarkers for clinical CTCL monitoring...
October 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29028854/clinical-insights-and-emerging-strategies-in-managing-cutaneous-t-cell-lymphoma
#17
Christina C Patrone, Larisa J Geskin
<p>Mycosis Fungoides and Sézary Syndrome, the two most common types of Cutaneous T-Cell Lymphoma (CTCL), present many management challenges for dermatologists. Here, we provide a comprehensive review of up-to-date literature, guidelines, and expert clinical insights. We highlight the updates in the World Health Organization Classification of Cutaneous Lymphomas; we summarize the epidemiology, including a recently observed stabilization of increasing incidence of CTCL in the past decade and increased incidence in males, blacks, and veterans; we also provide the most recent updates on prognostic factors for CTCL...
May 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/29026585/plantar-keratoderma-of-s%C3%A3-zary-syndrome
#18
Konstantinos C Fragkos
Sézary syndrome is an extremely rare form of cutaneous T-cell lymphoma. It presents suddenly and is associated with a poor prognosis. Clinical recognition is crucial for the diagnostic process and initiation of appropriate treatment. Plantar keratoderma is usually pathognomonic for Sézary syndrome and clinicians should be alerted to its presence.
October 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28971139/double-positive-cd4-and-cd8-s%C3%A3-zary-syndrome
#19
Denis Miyashiro, Marina Passos Torrealba, Kelly Cristina Manfrere, Juliana Pereira, Maria Notomi Sato, José Antonio Sanches
No abstract text is available yet for this article.
November 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28961843/global-patterns-of-care-in-advanced-stage-mycosis-fungoides-sezary-syndrome-a-multicenter-retrospective-follow-up-study-from-the-cutaneous-lymphoma-international-consortium
#20
P Quaglino, M Maule, H M Prince, P Porcu, S Horwitz, M Duvic, R Talpur, M Vermeer, M Bagot, J Guitart, E Papadavid, J A Sanches, E Hodak, M Sugaya, E Berti, P Ortiz-Romero, N Pimpinelli, O Servitje, A Pileri, P L Zinzani, T Estrach, R Knobler, R Stadler, M T Fierro, S Alberti Violetti, I Amitay-Laish, C Antoniou, C Astrua, S Chaganti, F Child, A Combalia, S Fabbro, P Fava, V Grandi, C Jonak, E Martinez-Escala, M Kheterpal, E J Kim, C McCormack, T Miyagaki, D Miyashiro, S Morris, C Muniesa, V Nikolaou, G Ognibene, F Onida, S Osella-Abate, S Porkert, C Postigo-Llorente, C Ram-Wolff, S Ribero, K Rogers, M Sanlorenzo, R Stranzenbach, N Spaccarelli, A Stevens, D Zugna, A H Rook, L J Geskin, R Willemze, S Whittaker, R Hoppe, J Scarisbrick, Y Kim
Background: Advanced-stage mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival...
October 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
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