Division of Hematology, The Ohio State University Comprehensive Cancer Center, Columbus, OH.
Division of Hematology and Hematologic Malignancies, Huntsman Cancer Institute at the University of Utah, Salt Lake City, UT.
Blood Adv. 2024 Oct 22;8(20):5355-5364. doi: 10.1182/bloodadvances.2024013791.
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 patients with HGBL NOS 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% vs 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-germinal center B-cell subtype, and "dual-expresser lymphoma" 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 chimeric antigen receptor T-cell modalities, almost all patients with CNS recurrence ultimately succumbed to their disease.
目前对于未特指的高级别 B 细胞淋巴瘤（HGBL NOS）的中枢神经系统（CNS）风险知之甚少。因此，我们旨在描述 HGBL NOS 患者的基线 CNS 受累率、原发治疗后 CNS 复发风险以及管理策略。在这项多中心回顾性研究中，我们纳入了 2016 年至 2021 年期间在 20 家美国机构接受治疗的 160 例新诊断的 HGBL NOS 成年患者。11 例（7%）患者在诊断时存在基线 CNS 受累（脑膜 = 6 例，实质 = 4 例，两者均有 = 1 例）。仅 MYC 重排（OR = 3.5）和睾丸（男性）或女性盆腔（女性）受累（OR = 8.1）与基线 CNS 受累显著相关。有或无基线 CNS 受累的 HGBL NOS 患者的生存结局无显著差异（中位 PFS：有基线 CNS 受累组为 4 年，无基线 CNS 受累组为 2.4 年，P = 0.45）。3 年内 CNS 复发的累积发生率为 11%。基线 CNS 受累的患者风险最高（48.5% vs 无基线 CNS 受累患者的 8%），因此排除在 CNS 复发风险因素分析之外。CNS 复发的风险与血液或骨髓受累、CD5 表达、非生发中心 B 细胞亚型以及“双表达性淋巴瘤”表型显著相关，但高 CNS IPI 与之无关。复发 HGBL NOS 的预后较差，无论复发是全身性的还是局限于 CNS，且目前的挽救性治疗策略，包括自体移植和嵌合抗原受体 T 细胞治疗，几乎所有 CNS 复发患者最终都因疾病而死亡。
