Departments of Pathology and Dermatology, University of Michigan, Ann Arbor, Michigan.
Department of Dermatology, University of Michigan, Ann Arbor, Michigan.
Am J Hematol. 2019 Sep;94(9):1027-1041. doi: 10.1002/ajh.25577. Epub 2019 Jul 31.
Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of T-cell neoplasms involving the skin, the majority of which may be classified as Mycosis fungoides (MF) or Sézary syndrome (SS).
The diagnosis of MF or SS requires the integration of clinical and histopathologic data.
RISK-ADAPTED THERAPY: TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multi-disciplinary approach to treatment. For patients with disease limited to the skin, skin-directed therapies are preferred, as both disease-specific and overall survival for these patients is favorable. In contrast, patients with advanced-stage disease with significant nodal, visceral or blood involvement are generally approached with systemic therapies. These include biologic-response modifiers, histone deacetylase (HDAC) inhibitors, or antibody-based strategies, in an escalating fashion. In highly-selected patients, allogeneic stem-cell transplantation may be considered, as this may be curative in some patients.
皮肤 T 细胞淋巴瘤（CTCL）是一组异质性 T 细胞肿瘤，涉及皮肤，其中大多数可分为蕈样真菌病（MF）或 Sezary 综合征（SS）。
MF 或 SS 的诊断需要整合临床和组织病理学数据。
TNMB（肿瘤、淋巴结、转移、血液）分期仍然是 MF/SS 中最重要的预后因素，并为“风险适应”的多学科治疗方法奠定了基础。对于疾病仅限于皮肤的患者，首选皮肤靶向治疗，因为这些患者的疾病特异性和总体生存率都很好。相比之下，对于有明显淋巴结、内脏或血液受累的晚期疾病患者，通常采用全身治疗。这些治疗方法包括生物反应调节剂、组蛋白去乙酰化酶（HDAC）抑制剂或基于抗体的策略，呈递增方式。在高度选择的患者中，可考虑异基因干细胞移植，因为这在某些患者中可能是治愈性的。
