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 | _a86 | ||
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=fr&redirect-ssocas=7080 |
999 |
_c602809 _d602809 |