Department of Laboratories, Institute of Pathology, C.H.U.V. Lausanne, Lausanne, Switzerland.
Department of Clinical Hematology, C.H.U. of Liège, Liège, Belgium.
Ann Oncol. 2012 Dec;23(12):3143-3151. doi: 10.1093/annonc/mds150. Epub 2012 Jun 13.
Diffuse large B-cell lymphomas (DLBCLs) arising in specific extranodal sites have peculiar clinicopathologic features.
We analyzed a cohort of 187 primary Waldeyer's ring (WR) DLBCLs retrieved from GELA protocols using anthracyclin-based polychemotherapy.
Most patients (92%) had stage I-II disease. A germinal center B-cell-like (GCB) immunophenotype was observed in 61%, and BCL2 expression in 55%, of WR DLBCLs. BCL2, BCL6, IRF4 and MYC breakpoints were observed in, respectively, 3 of 42 (7%), 9 of 36 (25%), 2 of 26 (8%) and 4 of 40 (10%) contributive cases. A variable follicular pattern was evidenced in 30 of 68 (44%) large biopsy specimens. The 5-year progression-free survival (PFS) and the overall survival (OS) of 153 WR DLBCL patients with survival information were 69.5% and 77.8%, respectively. The GCB immunophenotype correlated with a better OS (P = 0.0015), while BCL2 expression predicted a worse OS (P = 0.037), an effect overcome by the GCB/non-GCB classification. Compared with matched nodal DLBCLs, WR DLBCLs with no age-adjusted international prognostic index factor disclosed a better 5-year PFS rate (77.5% versus 70.7%; P = 0.03).
WR DLBCLs display distinct clinicopathologic features compared with conventional DLBCLs, with usual localized-stage disease, common follicular features and a high frequency of GCB immunophenotype contrasting with a low rate of BCL2 rearrangements. In addition, they seem to be associated with a better outcome than their nodal counterpart.
特定结外部位发生的弥漫性大 B 细胞淋巴瘤（DLBCL）具有独特的临床病理特征。
我们分析了 GELA 方案中使用蒽环类药物为基础的联合化疗治疗的 187 例原发性咽淋巴环（WR）DLBCL 患者队列。
大多数患者（92%）患有Ⅰ-Ⅱ期疾病。61%的 WR DLBCL 观察到生发中心 B 细胞样（GCB）免疫表型，55%的 WR DLBCL 表达 BCL2。在 42 例有贡献的病例中，分别观察到 3 例（7%）BCL2、BCL6、IRF4 和 MYC 断点，36 例（25%）BCL6、26 例（8%）IRF4 和 40 例（10%）MYC 断点。30 例（44%）大活检标本中可见可变滤泡模式。有生存信息的 153 例 WR DLBCL 患者的 5 年无进展生存（PFS）和总生存（OS）分别为 69.5%和 77.8%。GCB 免疫表型与更好的 OS 相关（P=0.0015），而 BCL2 表达预示着更差的 OS（P=0.037），但通过 GCB/非-GCB 分类可以克服这种影响。与匹配的结内 DLBCL 相比，无年龄调整国际预后指数（IPI）因素的 WR DLBCL 显示出更好的 5 年 PFS 率（77.5%比 70.7%；P=0.03）。
WR DLBCL 与常规 DLBCL 相比具有明显的临床病理特征，表现为常见的局限性疾病、常见的滤泡特征和高频率的 GCB 免疫表型，而 BCL2 重排率较低。此外，它们似乎与更好的预后相关，而不是与结内 DLBCL 相关。
