文献标题：Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project.
文献出处：blood 2011 1.26.
期刊影响因子：10.556
PMID: 21270441

国际外周T-细胞淋巴瘤研究计划目的在于通过协作更好地理解外周T-细胞淋巴瘤(PTCL)。共22个机构提交1314例病例的临床和病理材料。其中一个目标是分析340例非特异性PTCL (PTCL-NOS)患者的临床和病理特征。

患者平均年龄60岁，大多数患者（69%）处于疾病晚期。绝大多数患者 (87%)表现为结节性疾病，但是62%的患者出现结外疾病。

5年总生存率(OS)为32%，年无事件生存(FFS)仅20%。大多数患者 (80%)经包括蒽环霉素的联合化疗治疗后存活率没有提高。

国际预后指数(IPI)可预测OS和 FFS (p<0.001)。分别进行临床和病理学预测因素多变量分析，当控制IPI,证实体积较大 (>10 cm)，血小板减少(< 150 x 109/L)，和高数量的转移性肿瘤细胞可以作为不良存活预测信号，但是归根到底，仅后者有意义。

因此，可以使用IPI和单纯病理特征对新型和个体化疗法治疗的PTCL-NOS患者进行分类。

医脉通推荐英文摘要
blood 2011 1.26.DOI 10.1182/blood-2010-09-310342.

Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project

Dennis D. Weisenburger1, Kerry J. Savage,Nancy Lee Harris

（Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, United States）

The International Peripheral T-cell Lymphoma Project is a collaborative effort to better understand peripheral T-cell lymphoma (PTCL). A total of 22 institutions submitted clinical and pathological material on 1,314 cases. One objective was to analyze the clinical and pathological features of 340 cases of PTCL, not otherwise specified (PTCL-NOS). The median age of the patients was 60 years and the majority (69%) presented with advanced stage disease. Most patients (87%) presented with nodal disease, but extranodal disease was present in 62%. The 5-year overall survival (OS) was 32% and the 5-year failure-free survival (FFS) was only 20%. The majority of patients (80%) were treated with combination chemotherapy that included an anthracycline, but there was no survival advantage. The International Prognostic Index (IPI) was predictive of both OS and FFS (p<0.001). Multivariate analysis of clinical and pathological prognostic factors, respectively, when controlling for the IPI, identified bulky disease (>10 cm), thrombocytopenia (< 150 x 109/L), and a high number of transformed tumor cells (>70%) as adverse predictors of survival, but only the latter was significant in final analysis. Thus, the IPI and a single pathological feature could be used to stratify patients with PTCL-NOS for novel and risk-adapted therapies.