Giordano Antonio, Pagano Livio
Department of Hematology, Fondazione Policlinico Universitario Agostino Gemelli - IRCCS, Largo A. Gemelli, 8 I-00168 Rome, Italy.
Università Cattolica del Sacro Cuore, Largo A. Gemelli, 8 I-00168 Rome, Italy.
Mediterr J Hematol Infect Dis. 2022 Mar 1;14(1):e2022029. doi: 10.4084/MJHID.2022.029. eCollection 2022.
Cutaneous T-cell lymphomas are a heterogeneous group of T-cell neoplasms involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Mycosis fungoides (MF) is usually associated with an indolent clinical course and intermittent, stable, or slow progression of the lesions. Extracutaneous involvement (lymph nodes, blood, or less commonly other organs) or large cell transformation (LCT) may be seen in advanced-stage disease. Sezary syndrome (SS) is a rare leukemic subtype of CTCL characterized by significant blood involvement, erythroderma, and often lymphadenopathy. Although the early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy is often necessary to treat advanced-stage disease. Systemic therapy options have evolved in recent years with the approval of novel agents such as vorinostat, brentuximab vedotin, and mogamulizumab. This review aims to discuss the diagnosis and management of advanced-stages MF and SS.
皮肤T细胞淋巴瘤是一组异质性的T细胞肿瘤，累及皮肤，其中大多数可归类为蕈样肉芽肿（MF）或 Sézary 综合征（SS）。蕈样肉芽肿（MF）通常与惰性临床病程以及病变的间歇性、稳定或缓慢进展相关。晚期疾病可能会出现皮肤外受累（淋巴结、血液，或较少见的其他器官）或大细胞转化（LCT）。Sézary 综合征（SS）是CTCL的一种罕见白血病亚型，其特征为血液明显受累、红皮病，且常伴有淋巴结病。虽然早期疾病主要通过皮肤定向治疗可得到有效治疗，但晚期疾病通常需要全身治疗。近年来，随着伏立诺他、贝林妥欧单抗和莫加莫拉单抗等新型药物的获批，全身治疗方案不断发展。本综述旨在讨论晚期MF和SS的诊断与管理。
