高级别 B 细胞淋巴瘤 (HGBL)-NOS 在临床病理学和遗传学上与 DLBCL/HGBL-DH 比 DLBCL 更相似,Leukemia

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高级别 B 细胞淋巴瘤 (HGBL)-NOS 在临床病理学和遗传学上与 DLBCL/HGBL-DH 比 DLBCL 更相似
Leukemia ( IF 12.8 ) Pub Date : 2022-12-13 , DOI: 10.1038/s41375-022-01778-9
Shaoying Li ₁ , Lianqun Qiu ₁ , Jie Xu ₁ , Pei Lin ₁ , Chi Young Ok ₁ , Guilin Tang ₁ , Timothy J McDonnell ₁ , M James You ₁ , Mahsa Khanlari ₁ , Roberto N Miranda ₁ , L Jeffrey Medeiros ₁

Affiliation

未特指的高级别 B 细胞淋巴瘤 (HGBL-NOS) 很少见，并且缺乏针对这些肿瘤的数据。我们研究了 136 名 HGBL-NOS 患者的临床病理和遗传特征，并将其与 DLBCL/HGBL-DH（ n = 224，由第 5 版 WHO 定义）和 DLBCL（n = 217）患者进行比较。HGBL-NOS 患者具有与 DLBCL/HGBL-DH 患者相似的临床特征。43% 的 HGBL-NOS 中存在MYC重排 ( MYC -R)。采用与 DLBCL/HGBL-DH 患者相似的诱导方案，HGBL-NOS 患者的中位总生存期 (OS) 为 28.9 个月，与 DLBCL/HGBL-DH 相似 ( p = 0.48)，但低于 DLBCL患者 ( p = 0.03）。与 R-CHOP 相比，R-EPOCH 诱导与 OS 改善相关。MYC -R、淋巴瘤病史和高IPI是HGBL-NOS患者的独立不良预后因素。对 HGBL-NOS 病例子集进行的全转录组分析显示，与 DLBCL/HGBL-DH 相比，其特征更类似于 DLBCL/HGBL-DH；53% 的 HGBL-NOS 具有 DH 样特征 (DH-like-Sig)，并且富含MYC -R。DH 样 Sig+ HGBL-NOS 患者的 OS 比 DH 样 Sig 阴性患者较差 ( p = 0.04)。总之，HGBL-NOS 的临床病理特征和基因表达谱与 DLBCL/HGBL-DH 比 DLBCL 更相似。HGBL-NOS 病例经常携带MYC-R 并具有类似 DH 的 Sig+。与标准 R-CHOP 相比，HGBL-NOS 中的 R-EPOCH 诱导似乎与 OS 改善相关。

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High-grade B-cell lymphoma (HGBL)-NOS is clinicopathologically and genetically more similar to DLBCL/HGBL-DH than DLBCL

High-grade B-cell lymphoma, not otherwise specified (HGBL-NOS) is rare and data focused on these neoplasms is lacking. We studied the clinicopathologic and genetic features of 136 HGBL-NOS patients and compared them to patients with DLBCL/HGBL-DH (n = 224, defined by 5th Edition WHO) and DLBCL (n = 217). HGBL-NOS patients had clinical features similar to DLBCL/HGBL-DH patients. MYC rearrangement (MYC-R) was present in 43% of HGBL-NOS. With induction regimen similar to DLBCL/HGBL-DH patients, HGBL-NOS patients had a median overall survival (OS) of 28.9 months, similar to DLBCL/HGBL-DH (p = 0.48) but inferior to DLBCL patients (p = 0.03). R-EPOCH induction was associated with improved OS compared with R-CHOP. MYC-R, history of lymphoma, and high IPI were independent adverse prognostic factors in HGBL-NOS patients. Whole transcriptome profiling performed on a subset of HGBL-NOS cases showed a profile more similar to DLBCL/HGBL-DH than to DLBCL; 53% of HGBL-NOS had a DH-like signature (DH-like-Sig) and were enriched for MYC-R. DH-like-Sig+ HGBL-NOS patients had a poorer OS than DH-like-Sig-negative patients (p = 0.04). In conclusion, HGBL-NOS has clinicopathologic features and a gene expression profile more similar to DLBCL/HGBL-DH than to DLBCL. Cases of HGBL-NOS frequently carry MYC-R and have a DH-like-Sig+. R-EPOCH induction in HGBL-NOS appears associated with improved OS compared with standard R-CHOP.

更新日期：2022-12-14

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