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Sezary syndrome

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https://www.readbyqxmd.com/read/28611188/new-quantitative-features-for-the-morphological-differentiation-of-abnormal-lymphoid-cell-images-from-peripheral-blood
#1
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
#2
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
#3
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
#4
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
#5
Thomas Aagaard Rasmussen, James McMahon, J Judy Chang, Jori Symons, Michael Roche, Ashanti Dantanarayana, Afam Okoye, Bonnie Hiener, Sarah Palmer, Wen Shi Lee, Stephen 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 non-malignant cells were quantified by flow cytometry. HIV-DNA in total CD4+ T-cells, in sorted malignant and non-malignant CD4+ T-cells was quantified by PCR and clonal expansion of HIV-DNA assessed by full-length next-generation sequencing...
May 16, 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
#6
Valeria Behle, Tanja von Braunmühl, Cyrus Sayehli, Anja Gesierich, Matthias Goebeler, Eva Geissinger, Marion Wobser
No abstract text is available yet for this article.
May 17, 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
#7
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
#8
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
https://www.readbyqxmd.com/read/28441377/cd4-positive-t-cell-large-granular-lymphocytosis-mimicking-sezary-syndrome-in-a-patient-with-mycosis-fungoides
#9
Ling Zhang, Magali Van den Bergh, Lubomir Sokol
A white woman aged 65 years presented with a macular, nonscaly, nonpruritic, erythematous lesion on her right breast. Test results revealed histological features similar to lichenoid dermatitis and early-phase primary cutaneous T-cell lymphoma with a subtype of mycosis fungoides (MF). Despite topical therapy with steroids, her skin disease continued to progress, so she underwent polymerase chain reaction and gene mutation testing. Two missense mutations were detected. The overall findings supported a diagnosis of co-occurring, CD4-positive large granular lymphocytosis and stage IA MF...
April 2017: Cancer Control: Journal of the Moffitt Cancer Center
https://www.readbyqxmd.com/read/28423383/remarkable-advances-in-the-management-of-mycosis-fungoides-and-the-sezary-syndrome
#10
Richard T Hoppe
No abstract text is available yet for this article.
2017: Oncology Research and Treatment
https://www.readbyqxmd.com/read/28408397/the-il-31-il-31-receptor-axis-general-features-and-role-in-tumor-microenvironment
#11
REVIEW
Elisa Ferretti, Anna Corcione, Vito Pistoia
IL-31 is a recently identified cytokine with a well-defined role in the pathogenesis of pruritus. IL-31, whose production is induced by IL-4 and IL-33, binds a heterodimeric receptor (R) composed of the exclusive IL-31RA chain and the shared oncostatin M R. Signaling through the IL-31R involves the MAPK, PI3K/AKT and Jak/STAT pathways. Different variants and isoforms of IL-31RA with different signaling activities have been identified. IL-31 is produced predominantly by circulating Th2 lymphocytes and skin-homing CLA(+)CD45RO(+) T cells...
April 13, 2017: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/28400642/cd3-cd56-cd4-cd8-cd20-cd30-peripheral-t-cell-non-hodgkin-s-lymphoma-a-rare-case-report
#12
Ashish Jagati, Bela J Shah, Sonal Tibrewal, Trusha Gajjar
Cutaneous T-cell lymphoma (CTCL) commonly presents as mycosis fungoides or Sezary syndrome, both having CD4 positivity. A subset of CTCL which lacks CD4 surface marker is classified as cutaneous γ and δ-T-cell lymphoma (CGD-TCL). Because of its rarity and inability to study large number of patients, the impact of immunophenotype on the clinical outcome of primary CTCL in patients is limited. We report a case of primary CGD-TCL in a 71-year-old male because of this rarity and to emphasize its aggressive nature...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28400635/oral-and-cutaneous-lymphomas-other-than-mycosis-fungoides-and-s%C3%A3-zary-syndrome-in-a-mexican-cohort-recategorization-and-evaluation-of-international-geographical-disparities
#13
Amparo Hernández-Salazar, Jorge Andrés García-Vera, Yann Charli-Joseph, Guadalupe Ortiz-Pedroza, Silvia Méndez-Flores, Rocío Orozco-Topete, Ana Lilia Morales-Leyte, Judith Domínguez-Cherit, Carmen Lome-Maldonado
BACKGROUND: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. AIMS: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28400633/new-targeted-treatments-for-cutaneous-t-cell-lymphomas
#14
Martine Bagot
Cutaneous T-cell lymphomas (CTCLs) represent a group of rare and heterogeneous diseases that are very difficult to treat at advanced stages. The development of monoclonal antibodies is a new hope for the treatment of these diseases. Alemtuzumab (Campath) is a humanized IgG1 kappa monoclonal antibody specific for CD52, an antigen expressed by most T and B lymphocytes. Alemtuzumab may frequently induce long-term remissions in patients with Sezary syndrome but high-dose treatments lead to severe cytopenia, immune depletion, and opportunistic infections...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28391645/hematopoietic-stem-cell-transplantation-in-advanced-cutaneous-t-cell-lymphoma
#15
Hiroshi Saruta, Chika Ohata, Ikko Muto, Taichi Imamura, Eijiro Oku, Koichi Ohshima, Koji Nagafuji, Takekuni Nakama
We retrospectively reviewed data pertaining to five patients with cutaneous T-cell lymphoma (CTCL) who had received hematopoietic stem cell transplantation (HSCT) between 2004 and 2015 at Kurume University Hospital, along with their clinical data until March 2016. For patients with advanced CTCL eligible for HSCT, autologous HSCT was performed when they responded well to chemotherapy, and allogeneic HSCT was selected for patients with advanced mycosis fungoides (MF)/Sézary syndrome (SS) and CTCL other than MF/SS with poor chemosensitivity...
April 9, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28365528/european-organisation-for-research-and-treatment-of-cancer-consensus-recommendations-for-the-treatment-of-mycosis-fungoides-s%C3%A3-zary-syndrome-update-2017
#16
REVIEW
Franz Trautinger, Johanna Eder, Chalid Assaf, Martine Bagot, Antonio Cozzio, Reinhard Dummer, Robert Gniadecki, Claus-Detlev Klemke, Pablo L Ortiz-Romero, Evangelia Papadavid, Nicola Pimpinelli, Pietro Quaglino, Annamari Ranki, Julia Scarisbrick, Rudolf Stadler, Liisa Väkevä, Maarten H Vermeer, Sean Whittaker, Rein Willemze, Robert Knobler
In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sézary syndrome (SS), the European Organisation for Research and Treatment of Cancer-Cutaneous Lymphoma Task Force (EORTC-CLTF) published in 2006 its consensus recommendations for the stage-adapted selection of management options for these neoplasms. Since then, the understanding of the pathophysiology and epidemiology of MF/SS has advanced, the staging system has been revised, new outcome data have been published and novel treatment options have been introduced...
May 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28340880/mycosis-fungoides-and-sezary-syndrome
#17
REVIEW
Francine M Foss, Michael Girardi
Mycosis fungoides and the Sezary syndrome (SS) are rare lymphomas of CD4(+) helper T cells. There is stagewise progression from patch/plaques to thicker tumor lesions/diffuse erythroderma. Blood involvement is a characteristic of SS. Outcomes are related to the extent of skin, blood, lymph node, and visceral organ involvement. Patients with limited patch and plaque disease are treated with skin-directed therapies. More advanced/refractory disease is treated with skin-directed therapies and oral or systemic immunomodulatory agents...
April 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28338261/extracorporeal-photopheresis-for-the-treatment-of-early-stage-mycosis-fungoides
#18
Daniel J Lewis, Madeleine Duvic
Extracorporeal photopheresis (ECP) has been used for nearly 30 years in the treatment of cutaneous T-cell lymphoma. However, current clinical practice largely reserves ECP for patients with late-stage mycosis fungoides (MF) or Sézary syndrome or for those who are refractory to other therapies. We briefly describe a 48-year-old male who experienced long-term complete remission of his patch MF disease with ECP, and we suggest a role for ECP in the treatment of early-stage MF given evidence of its efficacy, safety, and tolerability...
March 24, 2017: Dermatologic Therapy
https://www.readbyqxmd.com/read/28325251/interferon-induced-thrombotic-microangiopathy-tma-analysis-and-concise-review
#19
REVIEW
Ajay Kundra, Jen Chin Wang
Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome...
April 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28301507/tp53-alterations-in-primary-and-secondary-s%C3%A3-zary-syndrome-a-diagnostic-tool-for-the-assessment-of-malignancy-in-patients-with-erythroderma
#20
Audrey Gros, Elodie Laharanne, Marie Vergier, Martina Prochazkova-Carlotti, Anne Pham-Ledard, Thomas Bandres, Sandrine Poglio, Sabine Berhouet, Béatrice Vergier, Jean-Philippe Vial, Edith Chevret, Marie Beylot-Barry, Jean-Philippe Merlio
Recent massive parallel sequencing data have evidenced the genetic diversity and complexity of Sézary syndrome mutational landscape with TP53 alterations being the most prevalent genetic abnormality. We analyzed a cohort of 35 patients with SS and a control group of 8 patients with chronic inflammatory dermatoses. TP53 status was analyzed at different clinical stages especially in 9 patients with a past-history of mycosis fungoides (MF), coined secondary SS. TP53 mutations were only detected in 10 patients with either primary or secondary SS (29%) corresponding to point mutations, small insertions and deletions which were unique in each case...
2017: PloS One
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