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

[Sézary syndrome treated with extracorporeal photopheresis: Report of one case].
 

Author(s): Montserrat Molgó, Alejandra Jaque, Verónica Vial, Mauricio Ocqueteau, Jaime Pereira, Mayling Chang, Sergio González

Journal: Rev Med Chil. 2015 Nov;143(11):1449-58.

 

Sézary syndrome (SS) is an unusually aggressive T- cell lymphoma characterized by the triad of erythroderma, the presence of more than 1,000 Sézary cells in peripheral blood and lymphadenopathies. It is accompanied by generalized pruritus and poor quality of life. The management ...

Last Updated: 13 Jan 2016

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The Therapeutic Potential of AN-7, a Novel Histone Deacetylase Inhibitor, for Treatment of Mycosis Fungoides/Sezary Syndrome Alone or with Doxorubicin.
 

Author(s): Lilach Moyal, Nataly Feldbaum, Neta Goldfeiz, Ada Rephaeli, Abraham Nudelman, Michal Weitman, Nataly Tarasenko, Batia Gorovitz, Leah Maron, Shiran Yehezkel, Iris Amitay-Laish, Ido Lubin, Emmilia Hodak

Journal:

 

The 2 histone deacetylase inhibitors (HDACIs) approved for the treatment of cutaneous T-cell lymphoma (CTCL) including mycosis fungoides/sezary syndrome (MF/SS), suberoylanilide hydroxamic acid (SAHA) and romidepsin, are associated with low rates of overall response and high rates ...

Last Updated: 12 Jan 2016

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Genomic profiling of Sézary syndrome identifies alterations of key T cell signaling and differentiation genes.
 

Author(s): Linghua Wang, Xiao Ni, Kyle R Covington, Betty Y Yang, Jessica Shiu, Xiang Zhang, Liu Xi, Qingchang Meng, Timothy Langridge, Jennifer Drummond, Lawrence A Donehower, Harshavardhan Doddapaneni, Donna M Muzny, Richard A Gibbs, David A Wheeler, Madeleine Duvic

Journal: Nat. Genet.. 2015 Dec;47(12):1426-34.

 

Sézary syndrome is a rare leukemic form of cutaneous T cell lymphoma characterized by generalized redness, scaling, itching and increased numbers of circulating atypical T lymphocytes. It is rarely curable, with poor prognosis. Here we present a multiplatform genomic analysis of ...

Last Updated: 1 Dec 2015

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

Hematopoietic Stem Cell Transplant for Mycosis Fungoides and Sézary Syndrome.
 

Author(s): Pooja Virmani, Jasmine Zain, Steven T Rosen, Patricia L Myskowski, Christiane Querfeld

Journal: Dermatol Clin. 2015 Oct;33(4):807-18.

 

Mycosis fungoides (MF) and Sézary syndrome (SS) are common types of primary cutaneous T-cell lymphoma. Early-stage MF has a favorable prognosis and responds well to skin-directed regimens. Patients with advanced-stage MF, transformed MF, and SS are treated with combined systemic ...

Last Updated: 5 Oct 2015

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Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sézary Syndrome.
 

Author(s): John A Zic

Journal: Dermatol Clin. 2015 Oct;33(4):765-76.

 

Extracorporeal photopheresis (ECP) is an immunomodulating procedure that leads to an expansion of peripheral blood dendritic cell populations and an enhanced TH1 immune response in cutaneous T-cell lymphoma (CTCL). Because of its excellent side effect profile and moderate efficacy, ...

Last Updated: 5 Oct 2015

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New and established treatment options for mycosis fungoides and Sézary syndrome - an update.
 

Author(s): Susanne Dugas-Breit, Hans-Joachim Schulze, Christian Hallermann

Journal: J Dtsch Dermatol Ges. 2014 Jul;12(7):561-9.

 

At the time of diagnosis primary cutaneous lymphomas are limited to the skin. T-cell lymphomas represent at least two thirds of all primary cutaneous lymphomas with mycosis fungoides and Sézary syndrome being the most frequent entities. A precise staging based on clinical, histological, ...

Last Updated: 1 Jul 2014

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

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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

Stem Cell Transplant Therapy With Campath-1H for Treating Advanced Mycosis Fungoides and Sezary Syndrome
 

Status: Recruiting

Condition Summary: Mycosis Fungoides; Sezary Syndrome

 

Last Updated: 31 Aug 2016

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Study of Alemtuzumab to Treat Advanced Mycosis Fungoides/Sezary Syndrome
 

Status: Recruiting

Condition Summary: Mycosis Fungoides; Sezary Syndrome

 

Last Updated: 2 Apr 2007

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TSEB and Brentuximab for Treatment of Mycosis Fungoides & Sezary Syndrome
 

Status: Not yet recruiting

Condition Summary: Mycosis Fungoides; Sézary Syndrome

 

Last Updated: 30 Jun 2016

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