Department of Dermatology, Medical University of Vienna, Austria.
Department of Dermatology, Faculty of Biology and Medicine, University of Lausanne, Switzerland.
J Dtsch Dermatol Ges. 2021 Sep;19(9):1307-1334. doi: 10.1111/ddg.14610.
Mycosis fungoides (MF) and Sézary syndrome (SS) are primary cutaneous T-cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor-cell burden (Sézary cells) in the peripheral blood consistent with advanced-stage disease and a poor prognosis. In early-stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early-stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin-directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets.
蕈样肉芽肿（MF）和赛泽里综合征（SS）是原发性皮肤 T 细胞淋巴瘤（CTCL），其病因和发病机制尚未完全阐明。从概念上讲，MF 和 SS 被归类为源自不同辅助性 T 细胞亚群的不同实体。MF 是最常见的 CTCL 实体，而 SS 非常罕见。MF 临床上表现为斑块、斑块和/或肿瘤期，但也可发展为红皮病，这反过来又是 SS 的特征性表现。SS 的特征是外周血中存在可检测到的肿瘤细胞负担（Sézary 细胞），与晚期疾病和预后不良相一致。在 MF 的早期阶段，即主要形式，预后通常良好。然而，多达 30%的患者存在皮肤病变进展，最终可能导致内脏受累。MF 的组织学表现在早期可能不明显，因此仔细的临床病理相关性至关重要。MF/SS 的治疗取决于疾病阶段。治疗方案包括皮肤靶向和系统治疗。除了异基因造血干细胞移植（alloSCT）外，MF/SS 目前尚无治愈性治疗方法。因此，治疗方法以症状为导向，旨在减轻肿瘤负担并改善与健康相关的生活质量。然而，随着对新型治疗靶点的发现，CTCL 的治疗领域不断扩大。
