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Advanced Disease

a) Advanced / refractory cutaneous disease : Occasionally if disease is refractory to one of these topical therapies, multi-agent therapy may be recommended. This may include:

  • Combination of topical therapy
  • Addition of oral agents: acetretin 10-25 mg/ day

However, after exhaustion of above mentioned therapies, with progressive disease, systemic chemotherapy can be used (see below).

b) Systemic disease: occasionally patients present with systemic disease and will need to be on systemic chemotherapy. There is no clear evidence that one medication is superior than another in terms of efficacy. The usual order of use is:

  • chlorambucil/prednisone
  • gemcitabine
  • Bexarotene (see chemo protocol: ULYMFBEX)
  • other

 c) Special circumstances:

  • occasionally > 5% circulating tumour cells/ sezary cells are seen. These patients are usually treated as above. However, for patients with refractory disease, who have circulating tumour cells, Extra-Corporeal Photoimmunotherapy has been used. (See chemo protocol ULYMFECP)

  • refractory lesions/tumours can be treated with localized radiation therapy

Reference: 
EORTC consensus recommendations for the treatment of mycosis Fungoides/ Sezary Syndrome EJC 42(2006) p1014-1030


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