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

Disseminated cutaneous leishmaniasis in a patient with Sézary syndrome.
 

Author(s): Priscila Giavedoni, Ignasi Pau-Charles, José M Mascaró, Mercé Alsina-Gibert, Adriana García-Herrera, Teresa Estrach

Journal: J. Am. Acad. Dermatol.. 2014 Nov;71(5):e213-4.

 

Last Updated: 2 Dec 2014

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Results of an open-label multicenter phase 2 trial of lenalidomide monotherapy in refractory mycosis fungoides and Sézary syndrome.
 

Author(s): Christiane Querfeld, Steven T Rosen, Joan Guitart, Madeleine Duvic, Youn H Kim, Stephen W Dusza, Timothy M Kuzel

Journal: Blood. 2014 Feb;123(8):1159-66.

 

A phase 2 multicenter trial was performed to evaluate single-agent lenalidomide in advanced, refractory mycosis fungoides/Sézary syndrome. Thirty-two patients were enrolled with a median of 6 prior treatment regimens, including a median of 4 systemic therapies. Patients achieved ...

Last Updated: 21 Feb 2014

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Comprehensive analysis of PTEN status in Sezary syndrome.
 

Author(s): Cristina Cristofoletti, Maria Cristina Picchio, Cristina Lazzeri, Valeria Tocco, Elena Pagani, Antonella Bresin, Barbara Mancini, Francesca Passarelli, Antonio Facchiano, Enrico Scala, Giuseppe Alfonso Lombardo, Maria Cantonetti, Elisabetta Caprini, Giandomenico Russo, Maria Grazia Narducci

Journal: Blood. 2013 Nov;122(20):3511-20.

 

Sézary syndrome (SS) is an incurable leukemic variant of cutaneous T-cell lymphoma characterized by recurrent chromosomal alterations, among which, chromosome 10q deletion is very frequent. In this study, we investigated the PTEN status, on locus 10q23, in 44 SS patients; our findings ...

Last Updated: 15 Nov 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 9 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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Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome).
 

Author(s): Henry K Wong, Anjali Mishra, Timothy Hake, Pierluigi Porcu

Journal: Br. J. Haematol.. 2011 Oct;155(2):150-66.

 

Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of malignancies derived from skin-homing T cells. The most common forms of CTCL are Mycosis Fungoides (MF) and Sezary Syndrome (SS). Accurate diagnosis remains a challenge due to the heterogeneity of presentation and the ...

Last Updated: 6 Oct 2011

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Symptoms, Diagnosis, and Treatment

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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 and Sezary Syndrome; Stage IIB Mycosis Fungoides and Sezary Syndrome; Stage IIIA Mycosis Fungoides and Sezary Syndrome; Stage IIIB Mycosis Fungoides and Sezary Syndrome; Stage IVA Mycosis Fungoides and Sezary Syndrome; Stage IVB Mycosis Fungoides and Sezary Syndrome

 

Last Updated: 9 Mar 2015

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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: 8 Dec 2014

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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: 15 May 2014

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