Abstract：
Nodal peripheral T-cell lymphoma (PTCL) with a T follicular helper phenotype (PTCL-TFH) is a new type of PTCL. We aimed to define its clinical characteristics and prognosis compared to PTCL not otherwise specified (PTCL-NOS) and angioimmunoblastic T-cell lymphoma (AITL). This retrospective observational study included 175 patients diagnosed with PTCL between 2008 and 2013 in 13 Spanish sites. Patient diagnosis was centrally reviewed, and patients were reclassified according to the World Health Organization (WHO) 2016 criteria: 21 patients as PTCL-NOS, 55 as AITL and 23 as PTCL-TFH. Median follow-up was 56.07 months (95% CI 38.7-73.4). Progression-free survival (PFS) and overall survival (OS) were significantly higher in patients with PTCL-TFH than in those with PTCL-NOS and AITL (PFS, 24.6 months vs. 4.6 and 7.8 months, respectively, p = 0.002; OS, 52.6 months vs. 10.0 and 19.3 months, respectively, p < 0.001). Histological diagnosis maintained an independent influence on both PFS (hazard ratio [HR] 4.1 vs. PTCL-NOS, p = 0.008; HR 2.6 vs. AITL, p = 0.047) and OS (HR 5.7 vs. PTCL-NOS, p = 0.004; HR 2.6 vs. AITL, p = 0.096), regardless of the International Prognostic Index. These results suggest that PTCL-TFH could have more favourable features and prognosis than the other PTCL subtypes, although larger series are needed to corroborate these findings.
摘要：
具有T滤泡辅助表型的淋巴结外周T细胞淋巴瘤(PTCL-TFH)是一种新型的PTCL。我们旨在与未指定的PTCL（PTCL-NOS）和血管免疫母细胞性T细胞淋巴瘤（AITL）相比，确定其临床特征和预后。这项回顾性观察研究包括2008年至2013年在西班牙13个地点诊断为PTCL的175例患者。对患者的诊断进行了集中审查，根据世界卫生组织（WHO）2016年标准对患者进行重新分类：21例患者为PTCL-NOS，55为AITL，23为PTCL-TFH。中位随访时间为56.07个月(95%CI38.7-73.4)。PTCL-TFH患者的无进展生存期（PFS）和总生存期（OS）明显高于PTCL-NOS和AITL患者（PFS，24.6个月vs.4.6和7.8个月，分别,p=0.002;OS,52.6个月vs.10.0和19.3个月，分别,p<0.001)。组织学诊断对两种PFS均具有独立影响（风险比[HR]4.1与PTCL-NOS,p=0.008;HR2.6vs.AITL,p=0.047）和OS（HR5.7与PTCL-NOS,p=0.004;HR2.6vs.AITL,p=0.096),无论国际预后指数如何。这些结果表明，PTCL-TFH可能比其他PTCL亚型具有更有利的特征和预后。尽管需要更大的系列来证实这些发现。
