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Primary Cutaneous T-Cell Lymphomas: Mycosis Fungoides and Sezary Syndrome

  • Christiane QuerfeldEmail author
  • Jasmine Zain
  • Steven T. Rosen
Chapter
Part of the Cancer Treatment and Research book series (CTAR, volume 176)

Abstract

Mycosis fungoides and Sézary syndrome are the most common subtypes of all primary cutaneous lymphomas and represent complex diseases that require a multidisciplinary assessment by dermatologists, oncologists, and pathologists. Staging and work-up are critical to guarantee an optimal treatment plan that includes skin-directed and/or systemic regimens depending on the clinical stage, tumor burden, drug-related side effect profile, and patient comorbidities. However, there is no cure and patients frequently relapse, requiring repeated treatment courses for disease control. The study of the tumor microenvironment and molecular mechanisms of these rare neoplasms may assist in the development of new immune therapies providing promising treatment approaches tailored for patients with relapse/refractory disease.

Keywords

Cutaneous T-cell lymphoma Mycosis fungoides Sézary syndrome Clinical and pathologic features Molecular hallmarks Tumor microenvironment Treatment strategies Immunotherapies 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Christiane Querfeld
    • 1
    • 2
    • 3
    • 4
    Email author
  • Jasmine Zain
    • 2
    • 4
  • Steven T. Rosen
    • 2
    • 4
  1. 1.Division of DermatologyCity of HopeDuarteUSA
  2. 2.Department of Hematology/Hematopoietic Cell TransplantationDuarteUSA
  3. 3.Department of PathologyDuarteUSA
  4. 4.Toni Stephenson Lymphoma Center, City of Hope National Medical CenterDuarteUSA

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