scholarly journals Downregulation of Fas Gene Expression in Sézary Syndrome Is Associated with Promoter Hypermethylation

2010 ◽  
Vol 130 (4) ◽  
pp. 1116-1125 ◽  
Author(s):  
Christine L. Jones ◽  
E. Mary Wain ◽  
Chung-Ching Chu ◽  
Isabella Tosi ◽  
Rosalind Foster ◽  
...  
Keyword(s):  
Gene Expression ◽  
Promoter Hypermethylation ◽  
Sézary Syndrome ◽  
Sezary Syndrome

scholarly journals Gene Expression Comparison between Sézary Syndrome and Lymphocytic-Variant Hypereosinophilic Syndrome Refines Biomarkers for Sézary Syndrome

Cells ◽  
2020 ◽  
Vol 9 (9) ◽  
pp. 1992
Author(s):  
Andrea Moerman-Herzog ◽  
Syed J. Mehdi ◽  
Henry K. Wong
Keyword(s):  
Gene Expression ◽  
T Cell ◽  
Expression Profiles ◽  
Meta Analysis ◽  
Hypereosinophilic Syndrome ◽  
Diagnostic Delay ◽  
Gene Expression Profiles ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Gene Expression Studies

Sézary syndrome (SS), an aggressive cutaneous T-cell lymphoma (CTCL) with poor prognosis, is characterized by the clinical hallmarks of circulating malignant T cells, erythroderma and lymphadenopathy. However, highly variable clinical skin manifestations and similarities with benign mimickers can lead to significant diagnostic delay and inappropriate therapy that can lead to disease progression and mortality. SS has been the focus of numerous transcriptomic-profiling studies to identify sensitive and specific diagnostic and prognostic biomarkers. Benign inflammatory disease controls (e.g., psoriasis, atopic dermatitis) have served to identify chronic inflammatory phenotypes in gene expression profiles, but provide limited insight into the lymphoproliferative and oncogenic roles of abnormal gene expression in SS. This perspective was recently clarified by a transcriptome meta-analysis comparing SS and lymphocytic-variant hypereosinophilic syndrome, a benign yet often clonal T-cell lymphoproliferation, with clinical features similar to SS. Here we review the rationale for selecting lymphocytic-variant hypereosinophilic syndrome (L-HES) as a disease control for SS, and discuss differentially expressed genes that may distinguish benign from malignant lymphoproliferative phenotypes, including additional context from prior gene expression studies to improve understanding of genes important in SS.

scholarly journals Aberrant Expression of the Tyrosine Kinase Receptor EphA4 and the Transcription Factor Twist in Sézary Syndrome Identified by Gene Expression Analysis

2004 ◽  
Vol 64 (16) ◽  
pp. 5578-5586 ◽  
Author(s):  
Remco van Doorn ◽  
Remco Dijkman ◽  
Maarten H. Vermeer ◽  
Jacoba J. Out-Luiting ◽  
Elisabeth M. H. van der Raaij-Helmer ◽  
...  
Keyword(s):  
Gene Expression ◽  
Transcription Factor ◽  
Tyrosine Kinase ◽  
Expression Analysis ◽  
Gene Expression Analysis ◽  
Tyrosine Kinase Receptor ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Aberrant Expression

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

Oncotarget ◽  
2017 ◽  
Vol 8 (24) ◽  
pp. 39627-39639 ◽  
Author(s):  
Katarzyna Iżykowska ◽  
Grzegorz K. Przybylski ◽  
Claudia Gand ◽  
Floriane C. Braun ◽  
Piotr Grabarczyk ◽  
...  
Keyword(s):  
Gene Expression ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Fusion Transcripts ◽  
Altered Gene Expression ◽  
Altered Gene ◽  
Genetic Rearrangements

Oncogenomic analysis of mycosis fungoides reveals major differences with Sézary syndrome

Blood ◽  
2009 ◽  
Vol 113 (1) ◽  
pp. 127-136 ◽  
Author(s):  
Remco van Doorn ◽  
Marloes S. van Kester ◽  
Remco Dijkman ◽  
Maarten H. Vermeer ◽  
Aat A. Mulder ◽  
...  
Keyword(s):  
Gene Expression ◽  
T Cell ◽  
Mycosis Fungoides ◽  
Genetic Alterations ◽  
Comparative Genomic ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Chromosomal Alterations ◽  
T Cell Lymphomas ◽  
Skin Homing

Abstract Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, is a malignancy of mature, skin-homing T cells. Sézary syndrome (Sz) is often considered to represent a leukemic phase of MF. In this study, the pattern of numerical chromosomal alterations in MF tumor samples was defined using array-based comparative genomic hybridization (CGH); simultaneously, gene expression was analyzed using microarrays. Highly recurrent chromosomal alterations in MF include gain of 7q36, 7q21-7q22 and loss of 5q13 and 9p21. The pattern characteristic of MF differs markedly from chromosomal alterations observed in Sz. Integration of data from array-based CGH and gene-expression analysis yielded several candidate genes with potential relevance in the pathogenesis of MF. We confirmed that the FASTK and SKAP1 genes, residing in loci with recurrent gain, demonstrated increased expression. The RB1 and DLEU1 tumor suppressor genes showed diminished expression associated with loss. In addition, it was found that the presence of chromosomal alterations on 9p21, 8q24, and 1q21-1q22 was associated with poor prognosis in patients with MF. This study provides novel insight into genetic alterations underlying MF. Furthermore, our analysis uncovered genomic differences between MF and Sz, which suggest that the molecular pathogenesis and therefore therapeutic requirements of these cutaneous T-cell lymphomas may be distinct.

scholarly journals Novel approach to gene expression profiling in Sézary syndrome

2010 ◽  
Vol 163 (5) ◽  
pp. 1090-1094 ◽  
Author(s):  
R.G. Pomerantz ◽  
E.D. Mirvish ◽  
G. Erdos ◽  
L.D. Falo ◽  
L.J. Geskin
Keyword(s):  
Gene Expression ◽  
Gene Expression Profiling ◽  
Expression Profiling ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Novel Approach

Use of PLS3, Twist, CD158k/KIR3DL2, and NKp46 gene expression combination for reliable Sézary syndrome diagnosis

Blood ◽  
2013 ◽  
Vol 121 (8) ◽  
pp. 1477-1478 ◽  
Author(s):  
L. Michel ◽  
F. Jean-Louis ◽  
E. Begue ◽  
A. Bensussan ◽  
M. Bagot
Keyword(s):  
Gene Expression ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Syndrome Diagnosis

Mycosis fungoides? Sezary syndrome

1970 ◽  
Vol 101 (2) ◽  
pp. 244-246 ◽  
Author(s):  
H. H. Roenigk
Keyword(s):  
Mycosis Fungoides ◽  
Sézary Syndrome ◽  
Sezary Syndrome
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