Sézary syndrome: diagnostic criteria and therapeutic options

Semin Cutan Med Surg. 2000 Jun;19(2):100-8. doi: 10.1016/s1085-5629(00)80006-1.

Abstract

Sézary syndrome (SS) is a rare form of erythrodermic cutaneous T-cell lymphoma with hematological involvement and a poor prognosis. Therapies include photopheresis, with or without interferon, chemotherapy, and total skin electron beam therapy. The lack of any randomized studies makes it difficult to assess the effect of current therapy on survival. In addition, the different response rates reported for individual treatments may depend as much on the criteria used to define SS as the therapy itself. This article reviews the diagnostic tests that are needed to reliably diagnose SS and offers a critical analysis of current treatment options.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use*
  • Dermatitis, Exfoliative / etiology*
  • Diagnosis, Differential
  • Facies
  • Humans
  • Immunophenotyping
  • Lymphocyte Count
  • Neoplasm Staging
  • Photopheresis*
  • Prognosis
  • Sezary Syndrome / blood
  • Sezary Syndrome / complications
  • Sezary Syndrome / diagnosis*
  • Sezary Syndrome / pathology
  • Sezary Syndrome / therapy*
  • Skin Neoplasms / blood
  • Skin Neoplasms / complications
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / therapy*
  • Survival Analysis

Substances

  • Antineoplastic Agents