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Sezary syndrome

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https://www.readbyqxmd.com/read/28925057/serum-vascular-endothelial-growth-factor-a-levels-reflect-itch-severity-in-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#1
Minami Sakamoto, Tomomitsu Miyagaki, Hiroaki Kamijo, Tomonori Oka, Naomi Takahashi, Hiraku Suga, Ayumi Yoshizaki, Yoshihide Asano, Makoto Sugaya, Shinichi Sato
Angiogenesis is an important step to support progression of malignancies, including mycosis fungoides (MF) and Sézary syndrome (SS). Vascular endothelial growth factor (VEGF)-A, a key player in angiogenesis, is secreted by tumor cells of MF/SS and its expression levels in lesional skin correlated with disease severity. In this study, we examined serum VEGF-A levels in MF/SS patients. Serum VEGF-A levels were elevated in patients with erythrodermic MF/SS and the levels decreased after treatment. Importantly, serum VEGF-A levels positively correlated with markers for pruritus...
September 19, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28912774/therapeutic-antibodies-to-kir3dl2-and-other-target-antigens-on-cutaneous-t-cell-lymphomas
#2
REVIEW
Christian Schmitt, Anne Marie-Cardine, Armand Bensussan
KIR3DL2 is a member of the killer cell immunoglobulin-like receptor (KIR) family that was initially identified at the surface of natural killer (NK) cells. KIR3DL2, also known as CD158k, is expressed as a disulfide-linked homodimer. Each chain is composed of three immunoglobulin-like domains and a long cytoplasmic tail containing two immunoreceptor tyrosine-based inhibitory motifs. Beside its expression on NK cells, it is also found on rare circulating T lymphocytes, mainly CD8(+). Although the KIR gene number varies between haplotype, KIR3DL2 is a framework gene present in all individuals...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28872191/cutaneous-t-cell-lymphoma-2017-update-on-diagnosis-risk-stratification-and-management
#3
Ryan A Wilcox
DISEASE OVERVIEW: Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). DIAGNOSIS: The diagnosis of MF or SS requires the integration of clinical and histopathologic data. RISK-ADAPTED THERAPY: TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multi-disciplinary approach to treatment...
October 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28843295/novel-mutations-involving-nf-%C3%AE%C2%BAb-and-b-cell-signaling-pathways-in-primary-cutaneous-large-b-cell-lymphoma-leg-type-and-comparison-with-s%C3%A3-zary-syndrome
#4
Esther J Kim, Daniel J Lewis, Madeleine Duvic
Multiple genomic mutations, especially those involving the NF-κB pathway, have been characterized in primary cutaneous large B-cell lymphoma, leg type. However, its genomic profiling remains limited given its rarity. In a recent study, Mareschal et al. performed next-generation sequencing and whole-exome sequencing, identifying new driver genes while also confirming the role of myeloid differentiation primary response gene 88 in the molecular pathogenesis of the disease.
September 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28829215/monoclonal-antibodies-against-cutaneous-t-cell-lymphomas
#5
Mauro Alaibac
Cutaneous T-cell lymphomas (CTCLs) comprise of a group of rare and heterogeneous skin lymphoproliferative disorders derived from skin resident T cells. Treatment of CTCLs is based on skin-directed approaches and/or systemic therapies. Advanced CTCLs are difficult to treat with the currently available treatments as they generally fail to obtain prolonged clinical remission. Recent studies concerning the pathogenetic mechanisms that are operative in CTCL have provided additional potential therapeutic targets for the treatment of these disorders...
August 28, 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/28770285/-treatment-of-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#6
REVIEW
J P Nicolay, C Assaf
Adequate therapeutic management of cutaneous T-cell lymphoma (CTCL) requires the identification of the exact CTCL stage and entity within the current WHO classification. There is no curative therapy for CTCL yet, so that treatment currently aims at improving symptoms and quality of life as well as reducing relapse rates. The treatment has to be stage-adapted. Therapeutic options comprise skin-directed as well as systemic treatment. In early stages, phototherapy and local steroids are the first-line therapeutic options...
August 2, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28741231/large-cell-transformation-of-oral-mycosis-fungoides
#7
Ahmed S Sultan, Behzad Mostoufi, John C Papadimitriou, Rima Koka, John Basile, Rania H Younis
Mycosis fungoides (MF) accounts for approximately 50% of all primary cutaneous lymphomas. MF occurrence in the oral cavity is extremely rare with approximately 45 cases reported to date. We present a case of a 68 year-old man with a raised nodular lesion of the ventral tongue with clinical impression of irritational fibroma. Histopathologic and immunohistochemical (IHC) examination revealed a phenotype consistent with MF with large cell transformation in the context of Sezary syndrome. The histological diagnosis of oral MF requires a high index of suspicion and IHC panel to rule out large cell transformation...
July 24, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28709694/early-clinical-manifestations-of-s%C3%A3-zary-syndrome-a-multicenter-retrospective-cohort-study
#8
Aaron R Mangold, Agnieszka K Thompson, Mark D Davis, Ieva Saulite, Antonio Cozzio, Emmanuella Guenova, Emmilia Hodak, Iris Amitay-Laish, Ramon M Pujol, Mark R Pittelkow, Robert Gniadecki
BACKGROUND: Classic Sézary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease. OBJECTIVE: To describe the early clinical characteristics of patients with SS. METHODS: A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015. RESULTS: Erythroderma was the earliest recorded skin sign of SS in only 25...
October 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28695331/vorinostat-and-mithramycin-a-in-combination-therapy-as-an-interesting-strategy-for-the-treatment-of-s%C3%A3-zary-t-lymphoma-a-transcriptomic-approach
#9
R Ragheb, G Venton, R Chelbi, N Bonnet, T Le Treut, V Ivanov, C Mercier, P Poulin, N Beaufils, J Gabert, P Suchon, P Rihet, B Loriod, B Kahn-Perlès, Régis T Costello
SAHA (vorinostat) is a histone deacetylase inhibitor approved by the USA Food and Drug Administration (FDA) for treating advanced refractory cutaneous T cell lymphomas. As SAHA alters the expression of many genes under control of the Sp1 transcription factor, we examined the effect of its association with the FDA-approved anticancer antibiotic Mithramycin A (MTR, plicamycin), a competitive inhibitor of Sp1 binding to DNA. Sézary syndrome (SS) cells, expanded ex vivo from peripheral blood mononuclear cells of 4 patients, were tested for their sensitivity to the drugs regarding cytotoxicity and differential responsive gene expression...
July 10, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28694325/tcr-cxcr4-signaling-stabilizes-cytokine-mrna-transcripts-via-a-prex1-rac1-pathway-implications-for-ctcl
#10
Kimberly N Kremer, Brittney A Dinkel, Rosalie M Sterner, Douglas G Osborne, Dragan Jevremovic, Karen E Hedin
As with many immunopathologically driven diseases, the malignant T cells of cutaneous T-cell lymphomas (CTCLs), such as Sézary syndrome, display aberrant cytokine secretion patterns that contribute to pathology and disease progression. Targeting this disordered release of cytokines is complicated by the changing cytokine milieu that drives the phenotypic changes of CTCLs. Here, we characterize a novel signaling pathway that can be targeted to inhibit the secretion of cytokines by modulating either CXCR4 or CXCR4-mediated signaling...
August 24, 2017: Blood
https://www.readbyqxmd.com/read/28655598/annual-facility-treatment-volume-and-patient-survival-for-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#11
Benjamin H Kann, Henry S Park, Debra N Yeboa, Sanjay Aneja, Michael Girardi, Francine M Foss, Kenneth B Roberts, Lynn D Wilson
BACKGROUND: Management of mycosis fungoides and Sézary syndrome (MF/SS) is complex, and randomized evidence to guide treatment is lacking. The institutional treatment volumes for MF/SS might vary widely nationally and influence patient survival. PATIENTS AND METHODS: Using the National Cancer Database, we identified patients with a diagnosis of MF/SS from 2004 to 2011 in the United States who had received treatment at a reporting facility. The patients were grouped into quintiles according to their treatment facility's average annual treatment volume (ATV)...
June 24, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28648940/upregulation-of-inhibitory-signaling-receptor-programmed-death-marker-1-pd-1-in-disease-evolution-from-cutaneous-lymphoid-dyscrasias-to-mycosis-fungoides-and-sezary-s-syndrome
#12
Giang Huong Nguyen, Luke C Olson, Cynthia M Magro
BACKGROUND: Negative immunoregulatory checkpoints impede effective immune responses to tumor and reduce the action of anticancer agents. One such example is programmed death marker-1 (PD-1), an inhibitory signaling receptor expressed on activated and regulatory T-cells. PD-1 expression was reported in a few reports, but the expression profile of PD-1 and mycosis fungoides (MF) remains largely to be characterized. DESIGN: In this study, skin biopsies from 42 prelymphomatous T-cell dyscrasias (CLD), 9 Sezary's syndrome (SS), 103 MF, and 20 CD30+ lymphoproliferative diseases (LPD) were examined for PD-1 expression using immunohistochemistry...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28611188/new-quantitative-features-for-the-morphological-differentiation-of-abnormal-lymphoid-cell-images-from-peripheral-blood
#13
Laura Puigví, Anna Merino, Santiago Alférez, Andrea Acevedo, José Rodellar
AIMS: This work aims to propose a set of quantitative features through digital image analysis for significant morphological qualitative features of different cells for an objective discrimination among reactive, abnormal and blast lymphoid cells. METHODS: Abnormal lymphoid cells circulating in peripheral blood in chronic lymphocytic leukaemia, B-prolymphocytic leukaemia, hairy cell leukaemia, splenic marginal zone lymphoma, mantle cell lymphoma, follicular lymphoma, T-prolymphocytic leukaemia, T large granular lymphocytic leukaemia and Sézary syndrome, normal, reactive and blast lymphoid cells were included...
June 13, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28595473/cd4-cd26-lymphocytes-are-useful-to-assess-blood-involvement-and-define-b-ratings-in-cutaneous-t-cell-lymphoma
#14
Eric C Vonderheid, J Steve Hou
Bernengo et al. reported that >30% CD4(+)CD26(-) lymphocytes detect blood involvement in patients with mycosis fungoides (MF) and Sézary syndrome. In addition, the ISCL/EORTC suggested that this threshold might serve as a criterion for the B2 blood rating for staging. In this manuscript, we report our experience with measurement of CD4(+)CD26(-) and CD4(+)CD7(-) cells, Sézary cell counts, and aberrant T cells with diminished expression of CD2, CD3, or CD5 antigens. CD4(+)CD26(-) ≥30% occurred in 15 of 373 (4...
June 9, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28540671/multidisciplinary-management-of-mycosis-fungoides-s%C3%A3-zary-syndrome
#15
REVIEW
Sara Berg, Jennifer Villasenor-Park, Paul Haun, Ellen J Kim
PURPOSE OF REVIEW: Diagnosis and management of mycosis fungoides and Sézary syndrome (MF/SS) require accurate clinicopathological correlation and a multidisciplinary approach. We reviewed major advances in the field regarding diagnostic and prognostic tools as well as skin-directed therapies (SDTs) and systemic agents for MF/SS published in the past 2 years. RECENT FINDINGS: Improved technology (T-cell receptor high-throughput sequencing) and increased multicenter collaboration (Cutaneous Lymphoma International Consortium) have led to diagnostic/prognostic advances...
June 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28533196/transformation-of-mycosis-fungoides-sezary-syndrome-clinical-characteristics-and-prognosis
#16
Seçil Vural, Bengü Nisa Akay, Ayşenur Botsalı, Erden Atilla, Nehir Parlak, Aylin Okçu Heper, Hatice Şanlı
INTRODUCTION: Transformed mycosis fungoides (T-MF) is a rare variant of mycosis fungoides (MF) with an aggressive course. OBJECTIVES: In this study we aimed to describe characteristics MF/Sezary syndrome (SS) cases with transformation. MATERIALS AND METHODS: Patients diagnosed with transformed MF (T-MF) among MF/SS patients between 2000 and 2014 in a single center are evaluated retrospectively.Demographic, clinical, laboratory, immunophenotype features, response to treatment, survival and histopathologic features were analyzed...
May 23, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28514279/impact-of-alemtuzumab-on-hiv-persistence-in-an-hiv-infected-individual-on-antiretroviral-therapy-with-sezary-syndrome
#17
Thomas A Rasmussen, James McMahon, J Judy Chang, Jori Symons, Michael Roche, Ashanti Dantanarayana, Afam Okoye, Bonnie Hiener, Sarah Palmer, Wen Shi Lee, Stephen J Kent, Carrie Van Der Weyden, H Miles Prince, Paul U Cameron, Sharon R Lewin
OBJECTIVE: To study the effects of alemtuzumab on HIV persistence in an HIV-infected individual on antiretroviral therapy (ART) with Sezary syndrome, a rare malignancy of CD4 T cells. DESIGN: Case report. METHODS: Blood was collected 30 and 18 months prior to presentation with Sezary syndrome, at the time of presentation and during alemtuzumab. T-cell subsets in malignant (CD7-CD26-TCR-VBeta2+) and nonmalignant cells were quantified by flow cytometry...
August 24, 2017: AIDS
https://www.readbyqxmd.com/read/28512672/s%C3%A3-zary-syndrome-with-nodal-cd30-positive-manifestation-treated-with-brentuximab-vedotin-and-extracorporeal-photopheresis
#18
Valeria Behle, Tanja von Braunmühl, Cyrus Sayehli, Anja Gesierich, Matthias Goebeler, Eva Geissinger, Marion Wobser
No abstract text is available yet for this article.
August 31, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28489605/genetic-rearrangements-result-in-altered-gene-expression-and-novel-fusion-transcripts-in-s%C3%A3-zary-syndrome
#19
Katarzyna Iżykowska, Grzegorz K Przybylski, Claudia Gand, Floriane C Braun, Piotr Grabarczyk, Andreas W Kuss, Karolina Olek-Hrab, Armando N Bastidas Torres, Maarten H Vermeer, Willem H Zoutman, Cornelis P Tensen, Christian A Schmidt
Sézary syndrome (SS) is an aggressive, leukemic cutaneous T-cell lymphoma variant. Molecular pathogenesis of SS is still unclear despite many studies on genetic alterations, gene expression and epigenetic regulations. Through whole genome and transcriptome next generation sequencing nine Sézary syndrome patients were analyzed in terms of copy number variations and rearrangements affecting gene expression. Recurrent copy number variations were detected within 8q (MYC, TOX), 17p (TP53, NCOR1), 10q (PTEN, FAS), 2p (DNMT3A), 11q (USP28), 9p (CAAP1), but no recurrent rearrangements were identified...
June 13, 2017: Oncotarget
https://www.readbyqxmd.com/read/28466385/allogeneic-hematopoietic-stem-cell-transplantation-for-refractory-mycosis-fungoides-mf-and-sezary-syndrome-ss
#20
Erden Atilla, Pinar Ataca Atilla, Sinem Civriz Bozdag, Meltem Kurt Yuksel, Selami Kocak Toprak, Pervin Topcuoglu, Bengu Nisa Akay, Hatice Sanli, Gunhan Gurman, Muhit Ozcan
Cutaneous T cell lymphoma is a heterogeneous group of lymphoproliferative disorders with different clinical behavior and prognosis in which malignant T cells accumulate in the skin. In the relapsed/refractory stage, treatment strategy varies depending on clinical perspective. We retrospectively evaluated advanced stage relapse or refractory mycosis fungoides and Sezary syndrome patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at our hospital. The overall response rate was 25%, while the disease progressed and relapsed after transplant in 38% of patients...
May 2, 2017: International Journal of Hematology
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