Department of Dermatology and Cutaneous Biology, Sidney Kimmel College at Jefferson University Hospital, Thomas Jefferson University, 833 Chestnut Street, Suite 740, Philadelphia, PA, 19107, USA.
Department of Medical Oncology, Sidney Kimmel College at Jefferson University Hospital, Philadelphia, PA, USA.
Am J Clin Dermatol. 2019 Aug;20(4):571-578. doi: 10.1007/s40257-019-00431-z.
Mycosis fungoides is the most common form of cutaneous T cell lymphoma. Although normally presenting to physicians at an early stage and with an indolent course, mycosis fungoides can have a varied presentation. The National Comprehensive Cancer Network (NCCN) has created guidelines for the treatment and staging of mycosis fungoides. Although comprehensive, in practice these guidelines do not provide specific treatment regimens for lesions located in difficult locations and those recalcitrant to the recommended therapy. Because of this, suggestions based on the practices and decisions made at the multidisciplinary cutaneous lymphoma clinic at the Sidney Kimmel Cancer Center at Thomas Jefferson University, Philadelphia, PA, USA, are presented here. Lesions located in areas such as the face and intertriginous zones are often challenging to treat because first-line therapies are often inappropriate, with the locations increasing the possibility of side effects. Additionally, lesions located in the bathing suit distribution are often nonresponsive to first-line therapies for reasons still undetermined. Finally, although well-described, erythroderma secondary to mycosis fungoides is challenging to treat, with controversy surrounding various methods of control. This article both highlights difficult clinical scenarios and reviews the recommended treatment as provided by the NCCN guidelines and provides alternative therapy for lesions that are either difficult to treat because of the location or are recalcitrant to the recommended therapy. With suggestions for the apparent gaps in guidelines, providers can better treat patients who present with more complicated conditions.
蕈样肉芽肿是最常见的皮肤 T 细胞淋巴瘤。尽管蕈样肉芽肿通常在早期向医生就诊且呈惰性病程,但它的表现可能多种多样。美国费城托马斯·杰斐逊大学西德尼·金梅尔癌症中心的多学科皮肤淋巴瘤诊所基于实践和决策提出了一些建议,这些建议旨在补充国家综合癌症网络(NCCN)为蕈样肉芽肿治疗和分期制定的指南。尽管这些指南很全面,但在实践中,它们并没有为位于难以治疗部位的病变以及对推荐疗法有抗性的病变提供具体的治疗方案。
位于面部和皱褶部位等区域的病变通常难以治疗,因为一线疗法往往不适用,这些位置增加了出现副作用的可能性。此外,位于泳衣分布区域的病变对一线疗法常常没有反应,原因尚不清楚。最后,尽管蕈样肉芽肿继发的红皮病已有明确描述,但治疗仍然具有挑战性,各种控制方法存在争议。
本文既突出了困难的临床情况,又回顾了 NCCN 指南推荐的治疗方法,并为因位置而难以治疗或对推荐疗法有抗性的病变提供了替代治疗方法。通过对指南中明显空白的建议,医疗服务提供者可以更好地治疗出现更复杂情况的患者。