How is Sézary syndrome cutaneous T-cell lymphoma (CTCL) treated?

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

The treatment of Sézary syndrome should be predicated on disease burden and rapidity of progression. [20] Because infection is the major cause of death in patients with mycosis Sézary syndrome, one should attempt to preserve immune response, use immunomodulatory therapy before chemotherapy unless the disease burden or therapeutic failure requires otherwise, and consider combination therapy, particularly systemic immunomodulatory therapy plus skin-directed treatments, as a better option than monotherapy.

Because Staphylococcus infection may be associated with a disease flare, systemic antibiotics should be administered when in doubt to prevent life-threatening sepsis. Pruritus treatment is an important quality-of-life consideration and should not be neglected. [116] It can be so severe that special itch-targeted therapies may need to be devised.

Mogamulizumab, a CCR4-directed monoclonal antibody, was approved by the FDA in August 2018 for adults with Sézary syndrome or mycosis fungoides who have received at least 1 prior systemic therapy. [105]


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