Department of International Health, Immunology and Microbiology, University of Copenhagen, Copenhagen, Denmark.
Leukemia. 2012 Mar;26(3):424-32. doi: 10.1038/leu.2011.237. Epub 2011 Sep 9.
Cutaneous T-cell lymphoma (CTCL) is the term for diseases characterized by primary accumulation of malignant T cells in the skin. Patients with the two predominant clinical forms of CTCL called mycosis fungoides (MF) and Sézary syndrome (SS) characteristically develop severe immunodeficiency during disease progression and consequently patients with advanced disease frequently die of infections and not from the tumor burden. For decades, it has been suspected that the malignant T cells actively drive the evolving immunodeficiency to avoid antitumor immunity, yet, the underlying mechanisms remain unclear. The identification of a subset of highly immunosuppressive regulatory T cells (Tregs) triggered a variety of studies investigating if MF and SS are malignant proliferations of Tregs but seemingly discordant findings have been reported. Here, we review the literature to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency.
皮肤 T 细胞淋巴瘤（CTCL）是指以恶性 T 细胞在皮肤中主要积聚为特征的疾病。患有两种主要临床形式的 CTCL，即蕈样真菌病（MF）和赛泽里综合征（SS）的患者在疾病进展过程中会出现严重的免疫缺陷，因此，晚期疾病患者经常死于感染而不是肿瘤负担。几十年来，人们一直怀疑恶性 T 细胞会积极地推动不断发展的免疫缺陷，以避免抗肿瘤免疫，但潜在的机制仍不清楚。鉴定出一群高度免疫抑制性调节性 T 细胞（Tregs）引发了各种研究，以调查 MF 和 SS 是否是 Tregs 的恶性增殖，但似乎有报道称存在不一致的发现。在这里，我们回顾文献，以阐明 Tregs 在 MF 和 SS 中的作用，并讨论驱动免疫缺陷的潜在机制。
