Division of Hematology/Oncology, University of Michigan Comprehensive Cancer Center, Ann Arbor, Michigan, 48109-5948.
Am J Hematol. 2017 Oct;92(10):1085-1102. doi: 10.1002/ajh.24876.
Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders 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, expectant management or skin-directed therapies is 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 biologic-response modifiers or histone deacetylase inhibitors prior to escalating therapy to include systemic, single-agent chemotherapy. In highly-selected patients, allogeneic stem-cell transplantation may be considered, as this may be curative in some patients.
皮肤 T 细胞淋巴瘤是一组涉及皮肤的异质性 T 细胞淋巴增生性疾病，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“风险适应性”多学科治疗方法奠定了基础。对于疾病局限于皮肤的患者，首选期待治疗或皮肤靶向治疗，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，通常在进行包括全身、单药化疗在内的升级治疗之前，使用生物反应调节剂或组蛋白去乙酰化酶抑制剂进行治疗。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。
