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难治性晚期蕈样肉芽肿病例报告:使用莫加莫拉单抗成功治疗并改善患者生活质量

A case report of refractory advanced-stage mycosis fungoides: successful treatment and improved patient quality of life with mogamulizumab.

作者信息

Frischhut Nina, Nguyen Van Anh

机构信息

Department of Dermatology, Venereology, and Allergology, Medical University of Innsbruck, Innsbruck, Austria.

Department of Dermatology, Venereology, and Allergology, Medical University of Innsbruck, Anichstrasse 35, Innsbruck 6020, Austria.

出版信息

Ther Adv Hematol. 2024 Jul 30;15:20406207241260340. doi: 10.1177/20406207241260340. eCollection 2024.

Abstract

Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, is characterized by patches, plaques, and, in advanced stages, tumors and erythroderma. Early-stage MF may progress to advanced-stage disease in up to one-third of patients, conferring a worse prognosis and typically requiring systemic treatment for extracutaneous involvement. The most frequently reported signs and symptoms are pain, pruritus, scaling, and skin redness, with pruritus, the most bothersome symptom, exerting a profound impact on patients' health-related quality of life (HRQoL). These dermatologic signs and symptoms can overlap with those of other benign inflammatory dermatoses, such as eczema and psoriasis, and therefore, diagnostic delay is common in patients with MF. Moreover, identifying patients with features adversely affecting prognosis (e.g. large-cell transformation or folliculotropic variant) is a significant challenge. We report the case of a 75-year-old female patient who was misdiagnosed with eczema and then pityriasis rubra pilaris and consequently did not receive treatment for MF for 4 years. The patient was eventually correctly diagnosed with MF [stage IIIB (T4 N1 M0 B1)] in September 2018. The patient received several systemic treatments; however, she did not respond to or tolerate the treatments. Due to lack of treatment response, in July 2021, she was initiated on mogamulizumab, an anti-CC chemokine receptor 4 antibody with demonstrated effectiveness and licensed approval for adults with MF/Sézary syndrome who have received one or more prior systemic therapies. Treatment rapidly led to a complete response in blood after 1 week and in skin after 4 months. Mogamulizumab was well tolerated by the patient, who also reported a significant improvement in her HRQoL. After 1 year in complete response, mogamulizumab was discontinued. This case highlights the need for accurate and early diagnosis of MF to initiate disease-specific treatment and the importance of considering patient HRQoL when treating this condition.

摘要

蕈样肉芽肿(MF)是皮肤T细胞淋巴瘤最常见的形式,其特征为斑片、斑块,在晚期表现为肿瘤和红皮病。高达三分之一的早期MF患者可能进展为晚期疾病,预后较差,通常需要针对皮肤外受累进行全身治疗。最常报告的体征和症状为疼痛、瘙痒、脱屑和皮肤发红,其中瘙痒是最困扰人的症状,对患者的健康相关生活质量(HRQoL)产生深远影响。这些皮肤体征和症状可能与其他良性炎症性皮肤病(如湿疹和银屑病)的体征和症状重叠,因此,MF患者中诊断延迟很常见。此外,识别具有不良预后特征(如大细胞转化或亲毛囊性变体)的患者是一项重大挑战。我们报告了一例75岁女性患者的病例,该患者最初被误诊为湿疹,后又被误诊为毛发红糠疹,因此4年未接受MF治疗。该患者最终于2018年9月被正确诊断为MF [IIIB期(T4 N1 M0 B1)]。患者接受了多种全身治疗;然而,她对这些治疗无反应或不耐受。由于缺乏治疗反应,2021年7月,她开始使用莫格利珠单抗治疗,这是一种抗CC趋化因子受体4抗体,已证明对接受过一种或多种先前全身治疗的成人MF/塞扎里综合征有效且已获得许可批准。治疗1周后血液中迅速出现完全缓解,4个月后皮肤出现完全缓解。患者对莫格利珠单抗耐受性良好,她还报告其HRQoL有显著改善。在完全缓解1年后,停用了莫格利珠单抗。该病例强调了准确早期诊断MF以启动针对性治疗的必要性,以及在治疗这种疾病时考虑患者HRQoL的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e6f/11292691/de9b09544f0f/10.1177_20406207241260340-fig1.jpg

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