A Clinicopathologic Study of Lennert Lymphoma and Possible Prognostic Factors The Importance of Follicular Helper T-cell Markers and the Association With Angioimmunoblastic T-cell Lymphoma
A Clinicopathologic Study of Lennert Lymphoma and Possible Prognostic Factors The Importance of Follicular Helper T-cell Markers and the Association With Angioimmunoblastic T-cell Lymphoma
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DOI：
10.1097/pas.0000000000000694
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发表时间：
2016-09-01
影响因子：
5.6
通讯作者：
Ohshima, Koichi
中科院分区：
文献类型：
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作者：
Kurita, Daisuke;Miyoshi, Hiroaki;Ohshima, Koichi
Lennert淋巴瘤（LeL）是外周T细胞淋巴瘤的一种变体，未另行说明。LeL是一种罕见的疾病，很少有临床病理学研究对其进行研究。在这里，我们分析了26例LeL患者的临床病理特征，以确定潜在的预后因素。CD 4、CD 8、CD 4/CD 8、TIA-1和颗粒酶B阳性的患者分别为21例（80.8%）、4例（15.4%）、1例（3.8%）、4例（15.3%）和0例（0.0%）。关于滤泡辅助性T细胞（T-FH）标志物，分别在14例（53.8%）、13例（50.0%）、1例（3.8%）和0例（0.0%）患者中观察到程序性细胞死亡-1（PD-1）、CXCL 13、CD 10和BCL 6阳性的肿瘤细胞。至少1种TFH细胞标志物（PD-1、CXCL 13、CD 10和/或BCL 6）阳性的患者被定义为TFH细胞标志物阳性（n=15），其预后比TFH细胞标志物阴性患者（n=11）更差（P=0.011）。TFH细胞标记阳性和标记阴性的LeL患者的临床病理特征没有显着差异。此外，TFH细胞标志物阳性的LeL患者和血管免疫母细胞性T细胞淋巴瘤（AITL）患者（n=42）之间的预后没有显着差异。然而，与AITL相比，TFH细胞标志物阳性的LeL与B症状、皮疹、高中危或高危国际预后指数值、滤泡树突状细胞网扩张、多形性浸润、透明细胞以及CD 10和BCL 6阳性的频率显著降低相关。虽然可能很难明确区分TFH细胞标志物阳性的LeL和AITL，但我们的研究结果表明，TFH细胞标志物可用于识别将经历不利结局的LeL患者。
Lennert lymphoma (LeL) is a variant of peripheral T-cell lymphoma, not otherwise specified. Few clinicopathologic studies have investigated LeL, which is a rare disease. Here, we analyzed the clinicopathologic features of 26 patients with LeL to identify potential prognostic factors. Neoplastic cells positive for CD4, CD8, CD4/CD8, TIA-1, and granzyme B were observed in 21 (80.8%), 4 (15.4%), 1 (3.8%), 4 (15.3%), and 0 (0.0%) patients, respectively. Regarding follicular helper T-cell (T-FH) markers, neoplastic cells positive for programmed cell death-1 (PD-1), CXCL13, CD10, and BCL6 were observed in 14 (53.8%), 13 (50.0%), 1 (3.8%), and 0 (0.0%) patients, respectively. Patients with positivity for at least 1 TFH cell marker (PD-1, CXCL13, CD10, and/or BCL6) were defined as being TFH cell marker-positive (n=15) and had a worse prognosis than TFH cell marker-negative patients (n=11) (P=0.011). Clinicopathologic characteristics did not differ significantly between TFH cell marker-positive and marker-negative LeL patients. Moreover, prognosis did not differ significantly between TFH cell marker-positive LeL patients and patients with angioimmunoblastic T-cell lymphoma (AITL) (n=42). Nevertheless, as compared with AITL, TFH cell marker-positive LeL was associated with significantly lower frequencies of B symptoms, skin rash, high-intermediate-risk or high-risk international prognostic index values, expanded follicular dendritic cell meshworks,polymorphic infiltrate, clear cells, and positivity for CD10 and BCL6. Although it may be difficult to definitively distinguish between TFH cell marker-positive LeL and AITL, our results suggest that TFH cell markers are useful for identifying LeL patients who will experience unfavorable outcomes.