Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine.
Memorial Sloan Kettering Cancer Center.
J Natl Compr Canc Netw. 2020 May;18(5):522-536. doi: 10.6004/jnccn.2020.0022.
Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma (CTCL), and Sézary syndrome (SS) is a rare erythrodermic and leukemic subtype of CTCL characterized by significant blood involvement. Although early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy is often necessary for the treatment of advanced-stage disease. Systemic therapy options have evolved in recent years with the approval of novel agents such as romidepsin, brentuximab vedotin, and mogamulizumab. These NCCN Guidelines Insights discuss the diagnosis and management of MF and SS (with a focus on systemic therapy).
蕈样肉芽肿（MF）是皮肤 T 细胞淋巴瘤（CTCL）最常见的亚型，而 Sézary 综合征（SS）是一种罕见的红皮病和白血病 CTCL 亚型，其特征为显著的血液受累。虽然早期疾病主要可以通过皮肤靶向治疗有效治疗，但对于晚期疾病的治疗，通常需要全身治疗。近年来，随着新型药物（如罗米地辛、 Brentuximab vedotin 和 mogamulizumab）的批准，全身治疗选择得到了发展。这些 NCCN 指南见解讨论了 MF 和 SS 的诊断和管理（重点是全身治疗）。
