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Sezary syndrome

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https://www.readbyqxmd.com/read/28340880/mycosis-fungoides-and-sezary-syndrome
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28296250/different-genetic-alteration-of-a20-in-a-s%C3%A3-zary-syndrome-case-with-v%C3%AE-2-j%C3%AE-22-t-cell-clone
#5
Lingling Zhou, Haitao Zheng, Xin Huang, Lihua Zhu, Suijing Wu, Chengwu Zeng, Lijian Yang, Shaohua Chen, Gengxin Luo, Xin Du, Yangqiu Li
BACKGROUND: The comprehensive genetic alterations underlying the pathogenesis of Sézary syndrome (SS) remains largely unknown. Previous studies showed that alterations of tumor necrosis factor-α-induced protein 3 gene (TNFAIP3; A20) are frequent in SS. In this study, we characterized the mutation and polymorphisms of A20 in a case with SS and compared with the genetic feature of A20 in T-cell acute lymphoblastic leukemia (T-ALL). METHODS: Using a novel approach based on the combination of fine-tiling array comparative genomic hybridization ( and ligation-mediated polymerase chain reaction (LM-PCR) to identify SS clone, the polymorphisms in the A20 gene (promoter, exons 2-9 [coding region] and 3'UTR) were detected by PCR and sequencing...
March 14, 2017: Asia-Pacific Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28271282/characterization-of-the-peripheral-neuropathy-associated-with-brentuximab-vedotin-treatment-of-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#6
Zachary A Corbin, Annie Nguyen-Lin, Shufeng Li, Ziba Rahbar, Mahkam Tavallaee, Hannes Vogel, Katrin A Salva, Gary S Wood, Youn H Kim, Seema Nagpal
Chemotherapy-induced peripheral neuropathy (CIPN) is common, frequently limits chemotherapy dosing, and negatively impacts quality of life. The National Cancer Institute Common Toxicity Criteria for Adverse Events (CTCAE), version 4.0, and the Total Neuropathy Score clinical version (TNSc) are both validated scores to quantify peripheral neuropathy (PN), with the TNSc being more sensitive to clinical changes. Mycosis fungoides and Sézary syndrome (MF/SS) are characterized by a chronic course, where current therapies are generally non-curative and treatment toxicities have the potential for significant lasting effects...
March 7, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28133821/total-skin-electron-therapy-as-treatment-for-epitheliotropic-lymphoma-in-a-dog
#7
Domenico Santoro, Lyndsay Kubicek, Bo Lu, William Craft, Julia Conway
BACKGROUND: Mycosis fungoides (MF) is an uncommon cutaneous neoplasm in dogs. Treatment options are limited. Total skin electron therapy (TSET) has been suggested as a possible therapy for canine MF. OBJECTIVE: To describe the use of TSET as palliative treatment for MF in a dog. RESULTS: An adult dog, previously diagnosed with nonepidermolytic ichthyosis, was presented with generalized erythroderma, alopecia and erosions. Histopathology revealed a densely cellular, well-demarcated, unencapsulated infiltrate extending from the epidermis to the mid-dermis compatible with MF...
January 29, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28119365/usefulness-of-kir3dl2-to-diagnose-follow-up-and-manage-the-treatment-of-s%C3%A3-zary-syndrome-patients
#8
Charlotte Hurabielle, Nicolas Thonnart, Caroline Ram-Wolff, Helene Sicard, Armand Bensussan, Martine Bagot, Anne Marie-Cardine
PURPOSE: KIR3DL2 is a recently discovered marker of the malignant clonal cell population in Sézary Syndrome (SS). We intended to evaluate the expression of KIR3DL2 on blood T-cells as a diagnostic, prognostic and follow-up marker of SS. EXPERIMENTAL DESIGN: 64 patients diagnosed with SS were included in this monocentric study. We collected the percentage of KIR3DL2+ cells among CD3+ T-cells, obtained by flow cytometry, and other classical diagnostic criteria for SS at diagnosis and during the follow-up...
January 24, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28107479/apoptosis-induction-and-gene-expression-profile-alterations-of-cutaneous-t-cell-lymphoma-cells-following-their-exposure-to-bortezomib-and-methotrexate
#9
Vassiliki Mpakou, Evangelia Papadavid, Frieda Kontsioti, Eugene Konsta, Miriam Vikentiou, Aris Spathis, Sotiris Papageorgiou, Diamantina Vasilatou, Konstantinos Gkontopoulos, Efthimia Mpazani, Petros Karakitsos, Dimitrios Rigopoulos, George Dimitriadis, Vasiliki Pappa
Mycosis fungoides (MF) and its leukemic variant Sézary syndrome (SS) comprise the majority of CTCL, a heterogenous group of non-Hodgkins lymphomas involving the skin. The CTCL's resistance to chemotherapy and the lack of full understanding of their pathogenesis request further investigation. With the view of a more targeted therapy, we evaluated in vitro the effectiveness of bortezomib and methotrexate, as well as their combination in CTCL cell lines, regarding apoptosis induction. Our data are of clinical value and indicate that the bortezomib/methotrexate combinational therapy has an inferior impact on the apoptosis of CTCL compared to monotherapy, with bortezomib presenting as the most efficient treatment option for SS and methotrexate for MF...
2017: PloS One
https://www.readbyqxmd.com/read/28034470/-cutaneous-granular-bacteriosis-occurring-in-staphylococcus-aureus-septicaemia
#10
D Mermin, A-L Védie, M-L Jullie, A Fauconneau, M Beylot-Barry, A Pham-Ledard
BACKGROUND: Herein we report a case of cutaneous granular bacteriosis, with discussion of the nosological setting of this entity based upon the clinical and histological findings. PATIENTS AND METHODS: A 62-year-old woman receiving methotrexate for Sezary syndrome was admitted for fever of 38.5̊C and overall impairment of her health. She presented a fistulous nodule on her right knee, and skin biopsy revealed a focus of ulcerated suppuration with quantities of Gram+ and Grocott+ granules containing no filament, enclosed by eosinophilic matter (Splendore-Hoeppli phenomenon)...
December 26, 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28024703/a-new-molecular-paradigm-in-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#11
Kojo S J Elenitoba-Johnson, Ryan Wilcox
Mycosis Fungoides (MF) and Sézary Syndrome (SS) are clonal proliferations of mature T-cells manifesting as lymphoproliferative disorders in which the neoplastic cells show a strong propensity for skin-homing. While the predominant site of presentation in MF is the skin, the peripheral blood carries a significant tumor burden in Sézary Syndrome such that it resembles a "leukemic" disease. While the genetic basis of these diseases has been studied using different approaches in the previous years, recent genome-wide studies employing massively parallel sequencing techniques now offer new insights into the molecular pathogenesis of these diseases...
January 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28012574/s%C3%A3-zary-syndrome-without-erythroderma-a-review-of-16-cases-at-mayo-clinic
#12
Agnieszka K Thompson, Jill M Killian, Amy L Weaver, Mark R Pittelkow, Mark D P Davis
BACKGROUND: Sézary syndrome (SS) is characterized by erythroderma with leukemic involvement. In atypical SS, leukemic involvement is present without erythroderma. Little is known about the presentation, prognosis, and outcome in these patients. OBJECTIVE: We sought to describe our experience with patients with SS without erythroderma. METHODS: We retrospectively identified patients with SS, but without erythroderma, at Mayo Clinic from 1976 to 2010...
December 21, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28010897/brentuximab-vedotin-for-treatment-of-non-hodgkin-lymphomas-a-systematic-review
#13
REVIEW
Garrett K Berger, Ali McBride, Stephanie Lawson, Kelsey Royball, Seongseok Yun, Kevin Gee, Irbaz Bin Riaz, Ahlam A Saleh, Soham Puvvada, Faiz Anwer
BACKGROUND: Brentuximab vedotin (BV) is an antibody-drug conjucate (ADC) comprising a CD30-directed antibody, conjugated to the microtubule-disrupting agent MMAE via a protease cleavable linker. BV is FDA approved for use in relapsed classical Hodgkin lymphoma (HL) and relapsed systemic anaplastic large cell lymphoma (sALCL). There are multiple publications for its utility in other malignancies such as diffuse large B-cell lymphoma (DLBCL), mycosis fungoides (MF), Sézary syndrome (SS), T-cell lymphomas (TCL), primary mediastinal lymphoma (PMBL), and post-transplant lymphoproliferative disorders (PTLD)...
January 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/27984038/increased-expression-of-pls3-correlates-with-better-outcome-in-s%C3%A3-zary-syndrome
#14
Stéphanie E Boonk, Willem H Zoutman, Hein Putter, Caroline Ram-Wolff, Moritz Felcht, Claus-Detlev Klemke, Annamari Ranki, Pietro Quaglino, Sean Whittaker, Martine Bagot, Rein Willemze, Maarten H Vermeer
No abstract text is available yet for this article.
March 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27981789/comparison-of-abnormal-cell-flagging-of-the-hematology-analyzers-sysmex-xn-and-sysmex-xe-5000-in-oncohematologic-patients
#15
COMPARATIVE STUDY
J R Furundarena, M Sainz, A Uranga, L Cuevas, I Lopez, J Zubicaray, A Bizjak, N Robado, M Araiz
INTRODUCTION: Hematology analyzers should optimize flagging while minimizing false-negative results and unnecessary microscopic reviews. METHODS: We compared flagging performance of Sysmex XE-5000 and XN analyzers in oncohematologic patients. Differential counts were performed by Cellavision digital system (100 cells) and a hematologist (another 100 cells). RESULTS: First, we included 292 samples (86 with blasts): 28 acute lymphoblastic leukemia, 88 acute myeloid leukemia, 91 myelodysplastic syndromes, 45 chronic myeloproliferative neoplasms, and 40 chronic myelomonocytic leukemia...
February 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27968935/cutaneous-lymphoma-kids-are-not-just-little-people
#16
Katalin Ferenczi, Hanspaul S Makkar
Cutaneous T-cell lymphomas (CTCLs) are non-Hodgkin lymphomas that predominantly affect older patients. Onset of cutaneous lymphoma in childhood is rare, but it can present as early as the first decade of life. In both adults and children, the diagnosis of cutaneous lymphoma can be challenging because inflammatory dermatoses can mimic CTCL both clinically and histologically. The clinicopathologic manifestations can be similar in adults and younger individuals; however, differences in the prevalence of certain CTCL variants among age groups exist...
November 2016: Clinics in Dermatology
https://www.readbyqxmd.com/read/27897325/the-biomarker-landscape-in-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#17
REVIEW
Brittany Dulmage, Larisa Geskin, Joan Guitart, Oleg E Akilov
The practice of pre-emptive individualized medicine is predicated on the discovery, development and application of biomarkers in specific clinical settings. Mycosis fungoides and Sézary syndrome are the two most common type of cutaneous T-cell lymphoma, yet diagnosis, prognosis and disease monitoring remain a challenge. In this review, we discuss the current state of biomarker discovery in mycosis fungoides and Sézary syndrome, highlighting the most promising molecules in different compartments. Further, we emphasize the need for continued multicentre efforts to validate available and new biomarkers and to develop prospective combinatorial panels of already discovered molecules...
November 28, 2016: Experimental Dermatology
https://www.readbyqxmd.com/read/27889686/microrna-181-contributes-to-downregulation-of-samhd1-expression-in-cd4-t-cells-derived-from-s%C3%A3-zary-syndrome-patients
#18
Rebecca Kohnken, Karthik M Kodigepalli, Anjali Mishra, Pierluigi Porcu, Li Wu
Sézary syndrome (SS) is a rare subtype of cutaneous T-cell lymphoma (CTCL) that is characterized by aggressive spread of neoplastic CD4+ T-cells from the skin into the bloodstream with metastasis to visceral organs. The deoxynucleoside triphosphohydrolase SAMHD1 is highly expressed in normal CD4+ T-cells, while its expression is down-regulated in CD4+ T-cells from SS patients. MicroRNA (miR) dysregulation is an important epigenetic mechanism in the pathogenesis and progression of SS. MiR-181 has been shown to inhibit SAMHD1 expression in cell lines and was identified as an important prognostic biomarker in CTCL...
January 2017: Leukemia Research
https://www.readbyqxmd.com/read/27873737/erythroderma-and-non-hodgkin-t-cell-lymphoma-what-else-apart-from-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#19
Alessandro Pileri, Cinzia Pellegrini, Claudio Agostinelli, Vieri Grandi, Annalisa Patrizi, Pier Luigi Zinzani, Nicola Pimpinelli
BACKGROUND: Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature. OBJECTIVES: We sought to describe PTCL-NOS cases characterised by erythrodermic dissemination to the skin...
February 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/27828640/alemtuzumab-in-refractory-s%C3%A3-zary-syndrome
#20
Carmen María Alcántara Reifs, Rafael Salido-Vallejo, Gloria María Garnacho-Saucedo, Sofía De la Corte-Sánchez, Alberto González-Menchen, Antonio Vélez García-Nieto
Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response...
September 2016: Anais Brasileiros de Dermatologia
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