What is the prognosis of Sézary syndrome cutaneous T-cell lymphoma (CTCL)?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Late-stage mycosis fungoides or Sézary syndrome is associated with declining immunocompetence. [47] Death most often results from systemic infection, especially with Staphylococcus aureus or Pseudomonas aeruginosa. [48] Secondary malignancies, such as higher-grade non-Hodgkin lymphoma, Hodgkin disease, colon cancer, and cardiopulmonary complications (eg, high output failure, comorbid cardiopulmonary disease) also contribute to mortality.

Median survival for patients with Sézary syndrome has been reported to be 2-4 years, [49] although the median survival was 2.9 years among patients defined by 2011 criteria for the disease. [20] The disease-specific 5-year survival rate has been reported to be 24%.


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