European Journal of Dermatology
MENUManagement experience of advanced-stage mycosis fungoides/Sézary syndrome: a retrospective study from Spanish haematology referral units Volume 30, issue 4, July-August 2020
- Key words: cutaneous T-cell lymphoma, mycosis fungoides, Sézary syndrome, allogeneic hematopoietic transplantation
- DOI : 10.1684/ejd.2020.3840
- Page(s) : 397-403
- Published in: 2020
Background: Advanced-stage mycosis fungoides/Sézary syndrome (aMF/SS) has a dismal outcome. The only curative treatment is allogeneic stem cell transplantation (allo-SCT) but this is limited to selected candidates, thus palliative therapy is the most frequent strategy. Objectives: To describe the characteristics of aMF/SS in cases referred to haematology units for advanced/palliative therapy. Materials and Methods: Data from 30 patients were collected from four centres, and descriptive statistics, frequencies and survival analyses were calculated. Results: Eighty-eight per cent of patients received systemic therapy. The median number of therapies was three (range: 1-9). Bexarotene (21%), CHOP-like chemotherapy (10%) and methotrexate (9%) were the more common treatments. The overall survival at a median follow-up of 28 months (range: 8-65 months) for aMF/SS was 56.9%. Survival probability was more favourable for MF (p < 0.02). Nine patients received allo-SCT. Half of the patients (56%) relapsed after allo-SCT but could be rescued with immunosuppression tapering, donor lymphocyte infusions and additional therapy (80%). Conclusion: There is significant heterogeneity in aMF/SS treatments. Survival is more favourable for MF compared to SS. Current chemoimmunotherapies are insufficient to control disease, making allo-SCT the best therapeutic approach in selected patients