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}
}

Figures and Tables from this paper

Mycosis fungoides and Sézary syndrome: clinical, immunological and molecular distinctions that suggest two different diseases

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.

Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

The literature is reviewed to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency.

Cutaneous T-Cell Lymphoma: A Review with a Focus on Targeted Agents

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.

Mycosis Fungoides and Related Lesions

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.

Sezary syndrome: a case study of cutaneous T-cell lymphoma.

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, Biology
    Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia
  • 2008
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

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

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.
...

References

SHOWING 1-10 OF 166 REFERENCES

Cutaneous T-cell lymphomas: the Sézary syndrome, mycosis fungoides, and related disorders.

It is suggested that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas", because the anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity.

Cutaneous T-cell lymphoma treatment using bexarotene and PUVA: a case series.

Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome.

In patients with erythrodermic mycosis fungoides and Sézary syndrome, the important prognostic factors are patient age at presentation, the overall stage, and peripheral blood involvement, and survival varies widely, depending on these variables.

Cutaneous T-cell lymphoma/leukemia

Opinion statementEffective treatment for cutaneous T-cell lymphomas (CTCL) requires an accurate and specific diagnosis based on the clinical presentation combined with evaluation of the

Immunopathogenesis and therapy of cutaneous T cell lymphoma.

Recent insights into the pathogenesis and immunobiology of MF/SS are summarized and how these have shaped current therapeutic approaches are summarized, in particular the growing emphasis on enhancement of host antitumor immune responses as the key to successful therapy.

Mycosis fungoides and Sezary syndrome are not associated with HTLV‐I infection: an international study

Molecular analyses were performed on 215 DNA samples by PCR amplification using HTLV‐I and ‐II gag, pol, env, pX and LTR specific primers, and Reverse transcriptase (RT) assay was performed in 26 cases using an RT‐PCR‐based method of high sensitivity.

Defining early mycosis fungoides.

Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas.

The hematologic criteria recommended for Sézary syndrome are intended to identify patients with a worse prognosis compared with the other E-CTCL subsets and consist of one or more of the following: an absolute SéZary cell count and a CD4/CD8 ratio of 10 or higher.

Mycosis fungoides shows concurrent deregulation of multiple genes involved in the TNF signaling pathway: an expression profile study.

Unsupervised hierarchic clustering has revealed 2 major subclasses of MF, one of which tends to include more aggressive-type MF cases including tumoral MF forms, and a 6-gene prediction model was constructed that is capable of distinguishing MF and ID cases with unprecedented accuracy.

Role of histology in providing prognostic information in mycosis fungoides

It appears unlikely that evaluation of any single biopsy specimen for the histologic parameters the authors studied is helpful in predicting the prognosis for a specific patient.
...