Department of Pathology, Centre Hospitalier Universitaire Sart Tilman, Liège, Belgium.
Best Pract Res Clin Haematol. 2012 Mar;25(1):13-28. doi: 10.1016/j.beha.2012.01.004. Epub 2012 Feb 7.
Peripheral T-cell lymphomas (PTCLs) are heterogeneous and uncommon malignancies characterized by an aggressive clinical course and a mostly poor outcome with current treatment strategies. The recent genome-wide molecular characterization of several entities has provided novel insights into their pathobiology and led to the identification of new biomarkers with diagnostic, prognostic or therapeutic implications for PTCL patients. Cell lineage and differentiation antigens (markers of γδ or NK lineage, of cytotoxicity, of follicular helper T cells) reflect the tumour's biological behaviour, and their detection in tissue samples may refine the diagnostic and prognostic stratification of the patients. Previously unrecognized gene rearrangements are being discovered (ITK-SYK translocation, IRF4/MUM1 and DUSP22 rearrangements), and may serve as diagnostic genetic markers. Deregulated molecules within oncogenic pathways (NF-κB, Syk, PDGFRα) and immunoreactive cell-surface antigens (CD30, CD52) have been brought to the fore as potential targets for guiding the development of novel therapies.
外周 T 细胞淋巴瘤（PTCLs）是一种异质性的罕见恶性肿瘤，其临床病程具有侵袭性，且目前的治疗策略大多预后较差。最近对几种实体进行的全基因组分子特征分析为其发病机制提供了新的见解，并确定了具有诊断、预后或治疗意义的新生物标志物，可用于 PTCL 患者。细胞谱系和分化抗原（γδ 或 NK 谱系标志物、细胞毒性标志物、滤泡辅助 T 细胞标志物）反映了肿瘤的生物学行为，在组织样本中检测这些标志物可以细化患者的诊断和预后分层。以前未被识别的基因重排（ITK-SYK 易位、IRF4/MUM1 和 DUSP22 重排）正在被发现，并且可以作为诊断遗传标志物。致癌途径中的失调分子（NF-κB、Syk、PDGFRα）和免疫反应性细胞表面抗原（CD30、CD52）已成为指导新型治疗方法发展的潜在靶点。
