PDF(Pubmed)
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：
霉菌病（MF），最常见的皮肤T细胞淋巴瘤,以补丁为特征，斑块,and,在高级阶段，肿瘤和红皮病。早期MF可能会在多达三分之一的患者中进展为晚期疾病，预后较差，通常需要对皮外受累进行全身治疗。最常报告的体征和症状是疼痛，瘙痒,缩放，皮肤发红,瘙痒，最麻烦的症状,对患者健康相关生活质量(HRQoL)产生深远影响。这些皮肤病学体征和症状可以与其他良性炎症性皮肤病重叠，比如湿疹和牛皮癣，因此,诊断延迟在MF患者中很常见。此外，识别具有对预后有不利影响的特征(例如大细胞转化或向叶酸变异)的患者是一项重大挑战.我们报告了一名75岁的女性患者，该患者被误诊为湿疹，然后被误诊为发痒糠疹，因此4年未接受MF治疗。患者最终于2018年9月被正确诊断为MF[IIIB期（T4N1M0B1）]。患者接受了几次全身治疗；然而，她对治疗没有反应或耐受。由于缺乏治疗反应，2021年7月，她开始服用莫加穆利珠单抗，一种抗CC趋化因子受体4抗体,在接受过一次或多次全身治疗的MF/Sézary综合征成人患者中已证实有效并获得批准.治疗迅速导致1周后血液和4个月后皮肤的完全反应。Mogamulizumab患者的耐受性良好，她的HRQoL也有显著改善。经过一年的完整响应，mogamulizumab停药.该病例强调了对MF进行准确和早期诊断以启动疾病特异性治疗的必要性，以及在治疗这种情况时考虑患者HRQoL的重要性。
