Long-term follow-up in 51 patients with mycosis fungoides and Sézary syndrome treated by interferon-alfa

Abstract

Although interferon-alfa (IFN-alpha) has proved effective in treating epidermotropic cutaneous T-cell lymphoma (ECTL), few studies have considered the follow-up of treated patients and whether complete remission was maintained. We studied 51 patients (one stage Ia, seven stage Ib, one stage IIa, 30 stage IIb, 11 stage III (Sézary syndrome) and one stage IV) who received low-dose IFN-alpha as monotherapy for ECTL (mean daily dose of IFN-alpha 2.7 x 106 units for 14.9 months), giving special consideration to the significance of My7 (CD13) antigen expression by epidermal basal cells in predicting the maintenance of complete remission. For a mean follow-up period of 43.4 months, the results showed 21 complete remissions, 13 partial remissions and 17 patients with stable or progressive disease. Twelve patients died during the follow-up (3-52 months). IFN-alpha led to an improved response in the early stages, with a greater number of complete remissions (P = 0.03) and partial remissions (P = 0.01). The mean time to complete remission was 4 months, regardless of clinical stage (P = 0.1). Of 21 patients in complete remission, 57% had a relapse within a mean period of 7.5 months. For patients maintained in complete remission, the mean period of response was 31 months. The length of complete remission was independent of clinical stage, and My7 antigen expression was not predictive of complete remission.

EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome

“…More recently, Zackheim and colleagues reported a retrospective study, in which 7 of 60 (12%) patients with patch/plaque MF (T2) attained a CR, and 13 (22%) achieved a PR. 105 Median time to treatment failure (TTF) was 15 months. The incidence and severity of adverse reactions to MTX therapy are related to the dose and frequency of administration.…”

“…Jumbou and colleagues reported longterm follow-up for a cohort of 51 patients with various stages of MF who were treated with IFN-a. 105 Most (35) patients had not received prior therapy. Of the 30 patients with stage IIB disease, 23 (77%) responded, including 10 (33%) CRs.…”

“…However, half of the patients in CR had disease relapse within 1 year. 105 12.3.1.2. TSEB and superficial X-irradiation.…”

Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

“…Interferon-alpha (i.e., interferon-alpha 2b), a type I interferon with immunomodulatory properties, has pleiotropic effects in CTCL and is associated with an overall response rate of 50-70% and a complete response rate of 20-30%, particularly in patients with limited-stage disease [285][286][287][288]. While often considered as second-line therapy for limitedstage CTCL, interferon-alpha, frequently at doses ranging from 3 to 10 million units daily to three times weekly, is a treatment to be considered in the first-line setting in patients with advanced-stage disease.…”

“…Furthermore, interferon-alpha may be successfully combined with a number of other therapeutic modalities, including PUVA, bexarotene, chemotherapy and ECP, which are frequently utilized in the management of these patients [289][290][291][292][293][294][295][296][297][298][299][300][301][302]. For example, in a cohort of 51 patients (42 of which had advancedstage disease) treated with single-agent, low-dose, interferon-alpha, responses were observed in 34 (67%), 21 (41%) of which were complete and maintained long-term in nine patients [288]. Similarly, in a cohort of 47 patients with stage III/IV disease, 89% of which had peripheral blood involvement, a response rate exceeding 80% was observed in those treated with a combination of ECP and interferonalpha [302].…”