高级别 B 细胞淋巴瘤，未另行说明：多机构系列中的中枢神经系统参与和结果。
High-Grade B-Cell Lymphoma, Not Otherwise Specified: CNS Involvement and Outcomes in a Multi-Institutional Series.
发表日期：2024 Aug 27
作者：
Narendranath Epperla, Adam Stephen Zayac, Daniel J Landsburg, Allison M Bock, Grzegorz S Nowakowski, Emily C Ayers, Mark Ryan Girton, Marie Hu, Amy K Beckman, Shaoying Li, L Jeffrey Medeiros, Julie E Chang, Habibe Kurt, Jose Sandoval-Sus, Mohammad Ali Ansari-Lari, Shalin K Kothari, Anna Kress, Mina L Xu, Pallawi Torka, Suchitra Sundaram, Stephen D Smith, Kikkeri N Naresh, Yasmin H Karimi, David A Bond, Andrew M Evens, Seema G Naik, Manali Kamdar, Bradley M Haverkos, Reem Karmali, Umar Farooq, Julie M Vose, Paul G Rubinstein, Amina Chaudhry, Adam J Olszewski
来源：
Blood Advances
摘要：
对于高级 B 细胞淋巴瘤的中枢神经系统 (CNS) 风险知之甚少，除非另有说明 (HGBL，NOS)。因此，我们试图描述 HGBL、NOS 的基线 CNS 受累率、主要治疗后 CNS 复发的风险以及管理策略。在这项多中心回顾性研究中，我们纳入了 2016 年至 2021 年间在 20 个美国机构接受治疗的 160 名新诊断 HGBL、NOS 的成年人。 11 名患者 (7%) 在诊断时有基线 CNS 受累（软脑膜 = 6，实质 = 4，两者 = 1）。基线中枢神经系统受累仅与 MYC 重排（OR=3.5）和睾丸（男性）或女性盆腔（女性）受累（OR=8.1）显着相关。基线 CNS 受累（中位 PFS=4 年）或无（中位 PFS=2.4 年）的 HGBL、NOS 患者之间的生存结果没有显着差异（p=0.45）。 3 年中枢神经系统复发的累积发生率为 11%。基线中枢神经系统受累的患者风险最高（基线中枢神经系统受累的患者为 48.5%，而基线无中枢神经系统受累的患者为 8%），并且被排除在中枢神经系统复发的危险因素分析之外。 CNS 复发风险与血液或骨髓受累、CD5 表达、非 GCB 亚型和 DEL 表型显着相关，但高 CNS-IPI 则不然。无论复发是全身性的还是仅限于中枢神经系统，复发的HGBL、NOS的预后都很差，并且利用目前可用的挽救策略，包括自体移植和CAR T细胞模式，几乎所有中枢神经系统复发的患者最终都死于疾病。 。这些患者代表了未满足的需求，应优先考虑实验方法。版权所有 © 2024 美国血液学会。
Little is known about the central nervous system (CNS) risk in high-grade B-cell lymphoma, not otherwise specified (HGBL, NOS). Hence, we sought to describe the rates of baseline CNS involvement, risk of CNS recurrence after primary therapy, and management strategies in HGBL, NOS. In this multicenter retrospective study, we included 160 adults with newly diagnosed HGBL, NOS treated between 2016 and 2021 at 20 US institutions. Eleven patients (7%) had baseline CNS involvement at diagnosis (leptomeningeal=6, parenchymal=4, and both=1). Baseline CNS involvement was significantly associated only with MYC rearrangement (OR=3.5) and testicular (in men) or female pelvic (in women) involvement (OR=8.1). There was no significant difference in survival outcomes between HGBL, NOS patients with (median PFS=4 years) or without (median PFS=2.4 years) baseline CNS involvement (p=0.45). The cumulative incidence of CNS recurrence at 3 years was 11%. Patients with baseline CNS involvement were at the highest risk (48.5% versus 8% for those without baseline CNS involvement) and were excluded from the risk factors analysis for CNS recurrence. The risk for CNS recurrence was significantly associated with blood or bone marrow involvement, CD5 expression, non-GCB subtype, and DEL phenotype, however, high CNS-IPI was not. The prognosis of relapsed HGBL, NOS was poor, regardless of whether recurrence was systemic or limited to the CNS, and with currently available salvage strategies, including autologous transplantation and CAR T-cell modalities, almost all patients with CNS recurrence ultimately succumbed to their disease. These patients represent an unmet need and should be prioritized for experimental approaches.Copyright © 2024 American Society of Hematology.