000 02373cam a2200265 4500500
005 20250121154622.0
041 _afre
042 _adc
100 1 0 _aNovelli, Silvana
_eauthor
700 1 0 _a Martín, Alejandro
_eauthor
700 1 0 _a Sánchez, Jose Javier
_eauthor
700 1 0 _a Espeso, Manuel
_eauthor
700 1 0 _a Mozos, Anna
_eauthor
700 1 0 _a Briones, Javier
_eauthor
245 0 0 _aManagement experience of advanced-stage mycosis fungoides/Sézary syndrome: a retrospective study from Spanish haematology referral units
260 _c2020.
500 _a87
520 _aBackground: 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
690 _amycosis fungoides
690 _aallogeneic hematopoietic transplantation
690 _aSézary syndrome
690 _acutaneous T-cell lymphoma
786 0 _nEuropean Journal of Dermatology | 30 | 4 | 2020-07-01 | p. 397-403 | 1167-1122
856 4 1 _uhttps://shs.cairn.info/revue-european-journal-of-dermatology-2020-4-page-397?lang=en&redirect-ssocas=7080
999 _c602810
_d602810