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

Downregulation of SAMHD1 expression correlates with promoter DNA methylation in Sézary syndrome patients.
 

Author(s): Suresh de Silva, Feifei Wang, Timothy S Hake, Pierluigi Porcu, Henry K Wong, Li Wu

Journal: J. Invest. Dermatol.. 2014 Feb;134(2):562-5.

 

Last Updated: 15 Jan 2014

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CD164 and FCRL3 are highly expressed on CD4+CD26- T cells in Sézary syndrome patients.
 

Author(s): Maria Wysocka, Andrew V Kossenkov, Bernice M Benoit, Andrea B Troxel, Elisha Singer, Andras Schaffer, Brian Kim, Tzvete Dentchev, Satoshi Nagata, Tomoko Ise, Louise C Showe, Alain H Rook

Journal: J. Invest. Dermatol.. 2014 Jan;134(1):229-36.

 

Sézary syndrome (SS) cells express cell surface molecules also found on normal activated CD4 T cells. In an effort to find a more specific surface marker for malignant SS cells, a microarray analysis of gene expression was performed. Results showed significantly increased levels ...

Last Updated: 19 Dec 2013

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Identification of multiple complex rearrangements associated with deletions in the 6q23-27 region in Sézary syndrome.
 

Author(s): Katarzyna Iżykowska, Mariola Zawada, Karina Nowicka, Piotr Grabarczyk, Floriane C M Braun, Martin Delin, Markus Möbs, Marc Beyer, Wolfram Sterry, Christian A Schmidt, Grzegorz K Przybylski

Journal: J. Invest. Dermatol.. 2013 Nov;133(11):2617-25.

 

The 6q23-27 region, recurrently deleted in Sézary syndrome (SS), was characterized at the molecular level in 13 SS patients and SS cell line SeAx. Using fine-tiling comparative genomic hybridization, deletions within the 6q23-27 region were detected in half of the samples (six patients ...

Last Updated: 16 Oct 2013

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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 8 free, full-text review articles on human participants. First 3 results:

A practical approach to accurate classification and staging of mycosis fungoides and Sézary syndrome.
 

Author(s): Bjorn Rhys Thomas, Sean Whittaker

Journal: Skin Therapy Lett.. 2012 Dec;17(10):5-9.

 

Cutaneous T-cell lymphomas are rare, distinct forms of non-Hodgkin's lymphomas. Of which, mycosis fungoides (MF) and Sézary syndrome (SS) are two of the most common forms. Careful, clear classification and staging of these lymphomas allow dermatologists to commence appropriate therapy ...

Last Updated: 11 Dec 2012

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Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update.
 

Author(s): Thamy Yamashita, Luciana Patricia Fernandes Abbade, Mariangela Esther Alencar Marques, Silvio Alencar Marques

Journal: An Bras Dermatol. ;87(6):817-28; quiz 829-30.

 

This paper reviews the diagnostic and classificatory concepts of mycosis fungoides and Sézary syndrome in light of the latest normative publications. It describes the great variability of the clinical expression of mycosis fungoides in its early stages as well as the histopathological ...

Last Updated: 30 Nov 2012

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Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer.
 

Author(s): Elise A Olsen, Sean Whittaker, Youn H Kim, Madeleine Duvic, H Miles Prince, Stuart R Lessin, Gary S Wood, Rein Willemze, Marie-France Demierre, Nicola Pimpinelli, Maria Grazia Bernengo, Pablo L Ortiz-Romero, Martine Bagot, Teresa Estrach, Joan Guitart, Robert Knobler, José Antonio Sanches, Keiji Iwatsuki, Makoto Sugaya, Reinhard Dummer, Mark Pittelkow, Richard Hoppe, Sareeta Parker, Larisa Geskin, Lauren Pinter-Brown, Michael Girardi, Günter Burg, Annamari Ranki, Maartan Vermeer, Steven Horwitz, Peter Heald, Steve Rosen, Lorenzo Cerroni, Brigette Dreno, Eric C Vonderheid, , ,

Journal: J. Clin. Oncol.. 2011 Jun;29(18):2598-607.

 

Mycosis fungoides (MF) and Sézary syndrome (SS), the major forms of cutaneous T-cell lymphoma, have unique characteristics that distinguish them from other types of non-Hodgkin's lymphomas. Clinical trials in MF/SS have suffered from a lack of standardization in evaluation, staging, ...

Last Updated: 20 Jun 2011

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Symptoms, Diagnosis, and Treatment

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

Pembrolizumab in Treating Patients With Relapsed or Refractory Stage IIB-IVB Mycosis Fungoides or Sezary Syndrome
 

Status: Recruiting

Condition Summary: Recurrent Mycosis Fungoides/Sezary Syndrome; Stage IIB Mycosis Fungoides/Sezary Syndrome; Stage IIIA Mycosis Fungoides/Sezary Syndrome; Stage IIIB Mycosis Fungoides/Sezary Syndrome; Stage IVA Mycosis Fungoides/Sezary Syndrome; Stage IVB Mycosis Fungoides/Sezary Syndrome

 

Last Updated: 17 Oct 2014

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Immunization Against Tumor Cells in Sezary Syndrome
 

Status: Recruiting

Condition Summary: Cutaneous T-Cell Lymphoma; Sezary Syndrome

 

Last Updated: 27 Sep 2007

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Effect of Neurokinin-1 Receptor (NK1R) Antagonism on Pruritus in Patients With Sezary Syndrome
 

Status: Recruiting

Condition Summary: Sezary Syndrome; Pruritus

 

Last Updated: 24 Apr 2014

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