Savage Kerry J
British Columbia Cancer Agency, 600 West 10th Avenue, Vancouver, British Columbia, Canada.
Blood Rev. 2007 Jul;21(4):201-16. doi: 10.1016/j.blre.2007.03.001. Epub 2007 May 18.
Peripheral T-cell lymphomas (PTCLs) are a biologically diverse and uncommon group of diseases. Compared to their B-cell counterparts, PTCLs remain largely unexplored and the optimal treatment ill-defined due to disease rarity and biological heterogeneity. With the notable exception of ALK-pos anaplastic large cell lymphoma (ALCL), the prognosis of most PTCL subtypes is extremely is poor with a 5 y overall survival of approximately 15-30% in most series. The international prognostic index has been useful in defining different risk groups within some PTCL subtypes, including PTCL unspecified (PTCLU). Attempts have been made to define disease subgroups within the biologically heterogeneous PTCLU based on T-helper chemokine receptor profile and/or gene expression profiling which may aid in tailoring new therapies. Future clinical trials are needed that focus specifically on PTCL to advance our understanding and define the optimal management in this disease.
外周T细胞淋巴瘤（PTCL）是一组生物学特性多样且罕见的疾病。与B细胞淋巴瘤相比，由于疾病罕见和生物学异质性，PTCL在很大程度上仍未得到充分研究，最佳治疗方法也尚不明确。除ALK阳性间变性大细胞淋巴瘤（ALCL）这一显著例外，大多数PTCL亚型的预后极差，在大多数系列研究中，5年总生存率约为15% - 30%。国际预后指数有助于在某些PTCL亚型中定义不同的风险组，包括未特指的PTCL（PTCLU）。人们已尝试基于T辅助趋化因子受体谱和/或基因表达谱在生物学异质性的PTCLU中定义疾病亚组，这可能有助于定制新的治疗方法。未来需要专门针对PTCL的临床试验，以增进我们对该疾病的了解并确定其最佳治疗方案。
