Ibatici Adalberto, Angelucci Emanuele, Massone Cesare
Hematology Unit and Cellular Therapies, San Martino Polyclinic Hospital, Genoa.
Dermatology Unit and Scientific Directorate, Galliera Hospital, Genoa, Italy.
Dermatol Reports. 2024 May 7;16(Suppl 2):9970. doi: 10.4081/dr.2024.9970.
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent non-Hodgkin lymphomas that comprise cutaneous T-cell lymphomas (CTCL), accounting for more than 70% of cases. Following the Tumor Lymph nodes Metastasis Blood system, disease staging is carried out, and within ten years, about thirty percent of patients in the early stages will have advanced disease. Plaques, folliculotropism, and age over 60 are risk factors for progression. A 5-year survival rate of less than 20% is associated with LCT in MF. Treatment requires an interdisciplinary approach; skin-directed therapies are available for early stages of the disease, but there are no curative options for advanced stages of the disease other than allogeneic stem cell transplantation. Because of their severe symptoms and poor treatment efficacy, patients in advanced stages have a lower quality of life and a lower chance of survival. In patients with CD30-expressing CTCL, Brentuximab Vedotin has demonstrated better response rates and progression-free survival (PFS); in advanced SS, mogamulizumab has significantly increased PFS. These findings emphasize the need to standardize prognostic factors and improve CTCL treatment.
蕈样肉芽肿(MF)和塞扎里综合征(SS)是最常见的非霍奇金淋巴瘤,属于皮肤T细胞淋巴瘤(CTCL),占病例的70%以上。按照肿瘤、淋巴结、转移、血液系统进行疾病分期,在十年内,约30%的早期患者会发展为晚期疾病。斑块、毛囊嗜性和60岁以上是疾病进展的危险因素。MF中局限性疾病(LCT)的5年生存率低于20%。治疗需要多学科方法;针对疾病早期有皮肤定向治疗,但除异基因干细胞移植外,疾病晚期没有治愈方案。由于晚期患者症状严重且治疗效果不佳,其生活质量较低,生存几率也较低。在表达CD30的CTCL患者中,维布妥昔单抗已显示出更好的缓解率和无进展生存期(PFS);在晚期SS中,莫格利珠单抗显著提高了PFS。这些发现强调了标准化预后因素和改善CTCL治疗的必要性。