Treatment of Stage III and Stage IV Mycosis Fungoides and Sézary Syndrome

Mycosis Fungoides

There is no curative therapy and no clear difference in overall survival (OS) among the treatment options for patients with stage III and stage IV disease.

The use of single alkylating agents has produced objective responses in 60% of patients, with a duration of less than 6 months. One of the alkylating agents (e.g., mechlorethamine, cyclophosphamide, or chlorambucil) or the antimetabolite methotrexate is the most frequently used. Single agents have not cured any patients, and insufficient data exist to determine whether these agents prolong survival. Combination chemotherapy is not definitely superior to single agents. Even in patients with stage IV disease, treatments directed at the skin may provide significant palliation.

A randomized study of 103 patients compared combined total-skin electron-beam radiation (TSEB) plus combination chemotherapy with conservation therapy consisting of sequential topical therapies.[1] In the latter group, combination chemotherapy was reserved for patients with symptomatic extracutaneous disease or disease that was refractory to topical therapies. Patients with any disease stage were eligible. Although the complete response rate was higher with combined therapy, toxic effects were considerably greater, and no difference was seen in disease-free survival (DFS) or OS between the two groups.[1][Level of evidence A1]

Sézary Syndrome

Sézary syndrome is a rare leukemic variant of cutaneous T-cell lymphoma. It is characterized by erythroderma, circulating Sézary cells with cerebriform nuclei, lymphadenopathy, and pruritus.[2] This condition typically progresses rapidly, with only a short duration of response to most therapies. A retrospective review of 176 patients with Sézary syndrome identified the following poor prognostic factors:[3]

Remissions attained by using extracorporeal photophoresis, interferon alfa, or retinoids may be followed by allogeneic stem cell transplant. In an anecdotal series of 16 patients with Sézary syndrome after allogeneic transplant, 9 were in complete remission after 4 years.[4]