儿童蕈样肉芽肿的治疗方法（ARMFUL 研究）：基于共识的建议。
Approach to Mycosis Fungoides in children (ARMFUL study): consensus-based recommendations.
发表日期：2024 Aug 22
作者：
Alex Zvulunov, Holly Neale, Jonah Stern, Pasqualina Santaguida, Amy Buros Stein, Mark Koh, Lawrence F Eichenfield, Joan Guitart, Matthias Goebeler, Julia Scarisbrick, Rein Willemze, Carrie C Coughlin, Renu George, Valeria Brazzelli, Márta Marschalkó, Irena Belousova, Christiane Querfeld, Martine Bagot, Jacek C Szepietowski, Evangelina Papadavid, Pietro Quaglino, Peter Hoeger, Pablo L Ortiz-Romero, Vasiliki Nikolaou, Reinhard Dummer, Phyu P Aung, Leslie Lawley, Kimberly D Morel, Bo Ngan, Mary Wain, Ana Gameiro, Rosa Mara Lacy-Niebla, Elena Pope
来源：
J Am Acad Dermatol
摘要：
尽管临床方面存在差异，但小儿蕈样肉芽肿 (MF) 的治疗仍根据成人指南进行推断。建议对于解决这一独特患者群体的独特挑战至关重要。该项目旨在为儿科 MF 管理得出共识建议。邀请了来自儿科皮肤病学、普通皮肤病学、皮肤病理学和儿科血液肿瘤学 (N=83) 的专家为共识建议做出贡献。该过程涉及三轮 eDelphi 轮，最后召开最终共识会议，使用修改后的名义小组技术来解决未解决的问题。共识包括比 TNMB 分期更多的临床严重程度测量：瘙痒、功能或美观损伤（例如手掌、脚底、生殖器）、质量生活影响、心理方面（例如尴尬、焦虑、抑郁）以及父母的焦虑。针对早期和晚期儿科 MF 的管理提出了十项建议。在选择超过疾病 I 期的治疗方法方面出现了分歧。这项跨国倡议旨在标准化最佳儿科 MF 管理，并成功产生了共识建议。需要对晚期儿科 MF 的结构化、前瞻性方案进行额外的工作。缺乏儿科血液学家、肿瘤学家和患者代表。建议记录扩展的临床严重程度和结果测量。满足晚期儿科 MF 对结构化方案的需求并实施系统性、前瞻性数据收集至关重要。版权所有 © 2024 American Academy of Dermatology, Inc. 由 Elsevier Inc. 出版。保留所有权利。
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.This project aims to derive consensus recommendations for pediatric MF management.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.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.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.Lack of pediatric hematologists-oncologists and patients' representatives.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..Copyright © 2024 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.