Phillips Adrienne A, Owens Colette, Lee Sangmin, Bhagat Govind
Division of Medical Oncology, Department of Medicine.
J Blood Med. 2011;2:119-29. doi: 10.2147/JBM.S8627. Epub 2011 Sep 12.
Peripheral T-cell lymphomas (PTCLs) comprise a rare and heterogeneous subset of non-Hodgkin's lymphomas (NHLs) that arise from post-thymic T-cells or natural killer (NK)-cells at nodal or extranodal sites. Worldwide, PTCLs represent approximately 12% of all NHLs and the 2008 World Health Organization (WHO) classification includes over 20 biologically and clinically distinct T/NK-cell neoplasms that differ significantly in presentation, pathology, and response to therapy. Because of the rarity and heterogeneity of these diseases, large clinical trials have not been conducted and optimal therapy is not well defined. Most subtypes are treated with similar combination chemotherapy regimens as used for aggressive B-cell NHL, but with poorer outcomes. New treatment combinations and novel agents are currently being explored for PTCLs and this review highlights a number of options that appear promising.
外周T细胞淋巴瘤(PTCLs)是一组罕见且异质性的非霍奇金淋巴瘤(NHLs),起源于胸腺后T细胞或自然杀伤(NK)细胞,发生于淋巴结或结外部位。在全球范围内,PTCLs约占所有NHLs的12%,2008年世界卫生组织(WHO)分类包括20多种生物学和临床特征各异的T/NK细胞肿瘤,其临床表现、病理及对治疗的反应差异显著。由于这些疾病罕见且异质性强,尚未开展大型临床试验,最佳治疗方案也未明确界定。大多数亚型采用与侵袭性B细胞NHL相似的联合化疗方案进行治疗,但疗效较差。目前正在探索针对PTCLs的新治疗组合和新型药物,本综述重点介绍了一些有前景的选择。