John Libbey Eurotext

European Journal of Dermatology


Management experience of advanced-stage mycosis fungoides/ Sézary syndrome: a retrospective study from Spanish haematology referral units Article à paraître


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1 Department of Hematology, Hospital de la Santa Creu i Sant Pau, José Carreras Leukemia Research Institute and IIB Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain
2 GELTAMO (Grupo Español de Linfoma y Trasplante de Médula Osea)
3 Department of Hematology, Hospital Universitario de Salamanca, IBSAL, CIBERONC, Salamanca, Spain
4 Department of Hematology, Hospital Morales Meseguer Murcia, Murcia, Spain
5 Department of Hematology, Hospital Carlos Haya, Málaga, Spain
6 Pathology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
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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