Mycosis fungoides and Sézary syndrome

@article{Hwang2008MycosisFA,
  title={Mycosis fungoides and S{\'e}zary syndrome},
  author={Samuel T. Hwang and John Edward Janik and Elaine S. Jaffe and Wyndham H Wilson},
  journal={The Lancet},
  year={2008},
  volume={371},
  pages={945-957}
}
Mycosis fungoides and Sézary syndrome are the most common of the cutaneous T-cell lymphomas, which are a heterogeneous group of neoplasms that affect the skin as a primary site. Although the aetiologies of mycosis fungoides and Sézary syndrome are unknown, important insights have been gained in the immunological and genetic perturbations that are associated with these diseases. Unlike some B-cell lymphomas, cutaneous T-cell lymphomas as a group are rarely if ever curable and hence need chronic… 
Cutaneous T-Cell Lymphoma: Mycosis Fungoides and Sézary Syndrome
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Gene expression analysis has uncovered many novel transcriptional abnormalities inMF/SS, hinting at epigenetic mechanisms potentially playing a role in the altered regulation, and these abnormal genes may serve as biomarkers in MF/SS diagnosis, provide insight into the pathogenesis of MF, and lead to new treatment approaches.
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Recent advances in the biological origin, immunophenotype, genetics and molecular profiles of mycosis fungoides and Sezary syndrome are surveyed, leading to a greater understanding of the two disease entities.
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TLDR
The literature is reviewed to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency.
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TLDR
The current landscape of targeted therapies in the treatment of CTCLs is examined, finding new monoclonal antibodies, checkpoint inhibitors, immunomodulatory agents, and small molecules are under investigation and may provide additional therapeutic options for those with advanced C TCL.
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TLDR
It has been proposed that ancillary molecular technologies with high sensitivities and specificities may be useful for more accurate diagnosis, staging, and prognostication of patients with mycosis fungoides.
Eruptive squamous cell carcinomas in an erythrodermic patient
TLDR
The greatest concern is that such erythroderma could represent an evolving cutaneous T-cell lymphoma (CTCL) (Mycosis fungoides [MF] or S ezary Syndrome) or a paraneoplastic finding indicative of another underlying malignancy.
Sezary syndrome: a case study of cutaneous T-cell lymphoma.
TLDR
A case study of a patient with Sezary syndrome is used to focus on the background of the syndrome, signs and symptoms, and new approaches to treatment.
Immunopathogenesis of mycosis fungoides/Sézary syndrome (cutaneous T-cell lymphoma).
  • H. Wong
  • Medicine
    Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia
  • 2008
TLDR
This review will focus on cytokine gene expression abnormalities in the clinical stages of the disease and discuss the relationship between the clinical and immunologic abnormalities to gain a better understanding of mechanisms important in the evolution of this disease.
Creating a Pathway to Best Clinical Practice for Cutaneous T-Cell Lymphoma
TLDR
Patient outcomes are dependent on knowledgeable dermatology nurses who can implement their understanding of MF and SS so as to improve skin integrity, assist with wound healing, palliate side effects from therapies, educate patients aboutMF and SS, and facilitate opportunities for additional support.
Diagnosis, prognosis and management of erythrodermic cutaneous T-cell lymphoma
TLDR
Improved patient selection and identification of appropriate conditioning regimens for reduced intensity allogeneic hematopoetic transplant are likely to improve survival, although a significant number of patients may not be fit for transplant because of advanced age and comorbidities.
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