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Sezary syndrome

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
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
#9
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
#10
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
#11
REVIEW
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
#12
REVIEW
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
#13
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
#14
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...
September 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28741231/large-cell-transformation-of-oral-mycosis-fungoides
#15
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
#16
MULTICENTER STUDY
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
#17
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...
October 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
#18
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
#19
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
#20
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
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