University Clinic for Dermatology, Venerology, Allergology and Phlebology, Johannes Wesling Medical Centre, UKRUB, University of Bochum, Minden, Germany.
Exp Dermatol. 2018 Oct;27(10):1078-1083. doi: 10.1111/exd.13701. Epub 2018 Jul 25.
Primary cutaneous T-cell lymphoma (CTCL) comprises the second most common group of extra-nodal non-Hodgkin's lymphoma. They represent incurable primary extra-nodal lymphomas of major T cells, uniformly present in the skin with 1%-2% risk of systemic dissemination in mycosis fungoides (MF), which represents the most common subtype of CTCL. In general, long-term antigen stimulation is thought, through key cytokine signalling pathways, to induce an inflammatory response with T-cell proliferation, leading to a clonal malignant T cell with continuous expansion. However, in recent years, using data harvested from high-throughput transcriptional profiling, substantial advances in the understanding of the molecular pathogenesis were made to understand the complex pathogenesis of CTCL. In this review, the actual data are summarised.
原发性皮肤 T 细胞淋巴瘤(CTCL)是第二大常见的结外非霍奇金淋巴瘤。它们代表了无法治愈的主要 T 细胞原发性结外淋巴瘤,1%-2%的蕈样肉芽肿(MF)存在系统性播散风险,MF 是 CTCL 最常见的亚型。一般来说,长期抗原刺激被认为通过关键细胞因子信号通路诱导炎症反应和 T 细胞增殖,导致克隆性恶性 T 细胞持续扩增。然而,近年来,通过高通量转录谱分析获得的数据,在理解 CTCL 的复杂发病机制方面取得了实质性进展。在这篇综述中,总结了实际数据。