Sezary syndrome

Common Name(s)

Sezary syndrome

Sezary syndrome is a type of cutaneous T-cell lymphoma (CTCL), which belongs to a larger group of disorders known as non-HodgkinÕs lymphomas. Sezary syndrome is characterized by a widespread red rash that may cover most of the body, the presence of specific malignant cells (Sezary cells) in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; outward turning of the eyelids (ectropion); palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly. The exact cause of cutaneous T-cell lymphomas is currently unknown. Treatment options vary depending on severity and signs and symptoms but may include topical chemotherapy, radiation therapy, photochemotherapy, use of retinoids, and chemotherapy. The prognosis is generally poor with a median survival between 2 and 4 years.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Sezary syndrome" for support, advocacy or research.

Cutaneous Lymphoma Foundation

The Cutaneous Lymphoma Foundation is an independent, nonprofit patient advocacy organization whose mission is supporting every person with cutaneous lymphoma by promoting awareness and education, advancing patient care and facilitating research.

Last Updated: 6 Jan 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Sezary syndrome" for support, advocacy or research.

Cutaneous Lymphoma Foundation

The Cutaneous Lymphoma Foundation is an independent, nonprofit patient advocacy organization whose mission is supporting every person with cutaneous lymphoma by promoting awareness and education, advancing patient care and facilitating research.

http://www.clfoundation.org

Last Updated: 6 Jan 2013

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Sezary syndrome" returned 188 free, full-text research articles on human participants. First 3 results:

Genetic rearrangements result in altered gene expression and novel fusion transcripts in Sézary syndrome.
 

Author(s): 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

Journal: Oncotarget. 2017 Jun;8(24):39627-39639.

 

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 ...

Last Updated: 31 Dec 1969

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TP53 alterations in primary and secondary Sézary syndrome: A diagnostic tool for the assessment of malignancy in patients with erythroderma.
 

Author(s): 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

Journal:

 

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 ...

Last Updated: 31 Dec 1969

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Characterization of the peripheral neuropathy associated with brentuximab vedotin treatment of Mycosis Fungoides and Sézary Syndrome.
 

Author(s): 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

Journal: J. Neurooncol.. 2017 05;132(3):439-446.

 

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 ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Sezary syndrome" returned 18 free, full-text review articles on human participants. First 3 results:

European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2017.
 

Author(s): 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

Journal: Eur. J. Cancer. 2017 05;77():57-74.

 

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 ...

Last Updated: 31 Dec 1969

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The biomarker landscape in mycosis fungoides and Sézary syndrome.
 

Author(s): Brittany Dulmage, Larisa Geskin, Joan Guitart, Oleg E Akilov

Journal: Exp. Dermatol.. 2017 08;26(8):668-676.

 

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 ...

Last Updated: 31 Dec 1969

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Romidepsin for the treatment of relapsed/refractory cutaneous T-cell lymphoma (mycosis fungoides/Sézary syndrome): Use in a community setting.
 

Author(s): Sunil A Reddy

Journal: Crit. Rev. Oncol. Hematol.. 2016 Oct;106():99-107.

 

Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of rare non-Hodgkin lymphomas that arise in the skin. In advanced stages, CTCL becomes systemic and is associated with poor prognosis. Diagnosis of CTCL and treatment of early-stage disease with topical therapies often occurs ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

TSEB and Brentuximab for Treatment of Mycosis Fungoides & Sezary Syndrome
 

Status: Recruiting

Condition Summary: Mycosis Fungoides; Sézary Syndrome

 

Last Updated: 25 Jun 2018

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Resminostat for Maintenance Treatment of Patients With Advanced Stage Mycosis Fungoides (MF) or Sézary Syndrome (SS)
 

Status: Recruiting

Condition Summary: Mycosis Fungoides; Sezary Syndrome; Lymphoma, T-Cell, Cutaneous

 

Last Updated: 28 May 2018

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Pembrolizumab and Interferon Gamma-1b in Treating Patients With Stage IB-IVB Relapsed or Refractory Mycosis Fungoides and Sezary Syndrome
 

Status: Recruiting

Condition Summary: Recurrent Mycosis Fungoides and Sezary Syndrome; Refractory Mycosis Fungoides; Stage IB Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage II Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IIA Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IIB Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage III Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IIIA Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IIIB Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IV Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IVA Mycosis Fungoides and Sezary Syndrome AJCC v7; Stage IVB Mycosis Fungoides and Sezary Syndrome AJCC v7

 

Last Updated: 9 Jul 2018

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