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Approach to Mycosis Fungoides in children (ARMFUL study): consensus-based recommendations

Articolo
Data di Pubblicazione:
2024
Abstract:
Background: Pediatric Mycosis Fungoides (MF) management extrapolates from adult guidelines, despite differing clinical aspects. Recommendations are essential to address unique challenges in this distinct patient group. Objective: This project aims to derive consensus recommendations for pediatric MF management. Methods: Experts from pediatric dermatology, general dermatology, dermato-pathology, and pediatric hematology-oncology (N=83) were invited to contribute to consensus recommendations. The process involved three eDelphi rounds, concluding with a final consensus meeting using a modified Nominal Group Technique for unresolved items. Results: Consensus included more clinical severity measures than TNMB staging: pruritus, functional or esthetic impairment (e.g., palms, soles, genitalia), quality of life impact, and psychological aspects (e.g., embarrassment, anxiety, depression), plus parental anxiety. Ten recommendations were made for managing early and advanced pediatric MF. Disagreement emerged in choosing therapies beyond stage I of the disease. Discussion: This multinational initiative aimed to standardize optimal pediatric MF management and successfully generated consensus recommendations. Additional work is needed for structured, prospective protocols in advanced-stage pediatric MF. Limitations: Lack of pediatric hematologists-oncologists and patients' representatives. Conclusion: Documentation of extended clinical severity and outcome measures is recommended. Addressing the need for structured protocols in advanced-stage pediatric MF and implementing systematic, prospective data collection is crucial..
Tipologia CRIS:
1.1 Articolo in rivista
Keywords:
consensus; mycosis fungoides; pediatric; severity and outcome measures
Elenco autori:
Zvulunov, Alex; Neale, Holly; Stern, Jonah; Santaguida, Pasqualina; Stein, Amy Buros; Koh, Mark; Eichenfield, Lawrence F; Guitart, Joan; Goebeler, Matthias; Scarisbrick, Julia; Willemze, Rein; Coughlin, Carrie C; George, Renu; Brazzelli, Valeria; Marschalkó, Márta; Belousova, Irena; Querfeld, Christiane; Bagot, Martine; Szepietowski, Jacek C; Papadavid, Evangelina; Quaglino, Pietro; Hoeger, Peter; Ortiz-Romero, Pablo L; Nikolaou, Vasiliki; Dummer, Reinhard; Aung, Phyu P; Lawley, Leslie; Morel, Kimberly D; Ngan, Bo; Wain, Mary; Gameiro, Ana; Lacy-Niebla, Rosa Mara; Pope, Elena
Autori di Ateneo:
BRAZZELLI VALERIA
Link alla scheda completa:
https://iris.unipv.it/handle/11571/1503855
Pubblicato in:
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
Journal
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