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.

Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.

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Advocacy and Support Organizations Health Questions Recommended Apps Scientific Literature Symptoms, Diagnosis, and Treatment Clinical Trial Information

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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General Support Organizations

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How do you compare to others with this condition?

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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

http://www.clfoundation.org

Last Updated: 6 Jan 2013

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General Support Organizations

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Relief is when you and the right researcher find each other

Finding the right clinical trial for Sezary syndrome can be challenging. However, with TrialsFinder (which uses the Reg4ALL database and privacy controls by Private Access), you can permit researchers to let you know opportunities to consider - all without revealing your identity.

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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 183 free, full-text research articles on human participants. First 3 results:

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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MicroRNA-181 contributes to downregulation of SAMHD1 expression in CD4+ T-cells derived from Sèzary syndrome patients.

Author(s): Rebecca Kohnken, Karthik M Kodigepalli, Anjali Mishra, Pierluigi Porcu, Li Wu

Journal: Leuk. Res.. 2017 Jan;52():58-66.

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 ...

Last Updated: 31 Dec 1969

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Alemtuzumab in refractory Sézary syndrome.

Author(s): Carmen María Alcántara Reifs, Rafael Salido-Vallejo, Gloria María Garnacho-Saucedo, Sofía De la Corte-Sánchez, Alberto González-Menchen, Antonio Vélez García-Nieto

Journal: An Bras Dermatol. ;91(5):642-644.

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 ...

Last Updated: 31 Dec 1969

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PubMed Article Results at NCBI: 183 article(s)

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 17 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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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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Global Patterns of Methylation in Sézary Syndrome Provide Insight into the Role of Epigenetics in Cutaneous T-Cell Lymphoma.

Author(s): Sean Whittaker

Journal: J. Invest. Dermatol.. 2016 Sep;136(9):1753-4.

van Doorn et al. have defined the DNA methylomes of Sézary cells based on a genome-wide methylation analysis using the Illumina 450K array platform (Illumina, San Diego, CA). Their results show aberrant DNA methylation patterns in CD4-enriched T cells from peripheral blood samples, ...

Last Updated: 31 Dec 1969

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PubMed Review Results at NCBI: 17 review(s)

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Symptoms, Diagnosis, and Treatment

This information is provided by Genetics Home Reference.

Full Results at Genetics Home Reference: 1674 matches

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

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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: 3 Oct 2017

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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: 19 Apr 2018

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A Trial Assessing the Effect of Pembrolizumab Combined With Radiotherapy in Patients With Relapsed, Refractory, Specified Stages of Cutaneous T-cell Lymphoma (CTCL) Mycosis Fungoides (MF)/Sezary Syndrome (SS)

Status: Not yet recruiting

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

Last Updated: 27 Dec 2017

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According to ClinicalTrials.gov there are currently 23 additional "open" studies for "Sezary syndrome" (open studies are recruiting volunteers) and 335 "Sezary syndrome" studies with "all" status. Visit ClinicalTrials.gov now to view them. Or alternatively, consider TrialsFinder for assistance:

Relief is when you and the right researcher find each other
Finding the right clinical trial for Sezary syndrome can be challenging. However, with TrialsFinder (which uses the Reg4ALL database and privacy controls by Private Access), you can permit researchers to let you know opportunities to consider - all without revealing your identity.

Learn More