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Sézary Syndrome

  • Amrita Goyal
  • Joi B. Carter
  • Aliyah R. Sohani
  • Jeffrey A. Barnes
  • Mai P. Hoang
  • Lyn McDivitt DuncanEmail author

Abstract

Sézary syndrome is a rare leukemia with a median survival of 3 years. Sézary syndrome patients comprise less than 5 % of all patients with cutaneous T-cell malignancies. Patients present with a triad of symptoms: erythroderma, generalized lymphadenopathy, and a clonal neoplastic proliferation of T cells with cerebriform nuclei in the peripheral blood. They commonly experience intense pruritus and significant derangements of cellular and humoral immunity. Diagnosis is based on clinical presentation, flow cytometric analysis, and histopathology. This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunophenotype, and differential diagnosis of Sézary syndrome. It closes with two clinical cases, including clinical images, histopathology, and flow cytometry.

Keywords

Sézary syndrome Th1 Th2 Erythroderma Lymphadenopathy Sézary cells CTCL Circulating memory T cells Central memory T cells Mycosis fungoides Large cell transformation Adult T-Cell Leukemia/Lymphoma (ATLL) 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Amrita Goyal
    • 1
  • Joi B. Carter
    • 2
  • Aliyah R. Sohani
    • 3
  • Jeffrey A. Barnes
    • 4
  • Mai P. Hoang
    • 5
  • Lyn McDivitt Duncan
    • 5
    Email author
  1. 1.Harvard-MIT Division of Health Sciences and TechnologyHarvard Medical SchoolBostonUSA
  2. 2.Department of DermatologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Hematopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  4. 4.Comprehensive Cutaneous Lymphoma Program, Department of MedicineMassachusetts General Hospital Cancer CenterBostonUSA
  5. 5.Dermatopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

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