Recent updates have detailed how patients with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) may be better risk stratified using prognostic scoring systems. Most patients with NLPHL present with early-stage disease and have an indolent disease course. To reflect these differences from classic Hodgkin lymphoma, nomenclature has been updated to recognize nodular lymphocyte-predominant B-cell lymphoma as an alternative to NLPHL. The Global NLPHL One Working Group have published their pivotal dataset in 2024 which challenges the prognostic significance of variant immunoarchitectural (IAP) patterns and proposes a new prognostic scoring system. Key identified prognostic factors include age >45 years, stage III-IV disease, hemoglobin <10.5 g/dL and splenic involvement. After multivariate analysis, variant IAP was not shown to be associated with inferior outcome. As most patients with NLPHL have excellent long-term survival, identifying patients where treatment de-escalation is appropriate will help to minimize toxicity. De-escalation strategies include observation after fully resected stage I disease, active surveillance, anti-CD20 antibody monotherapy, radiotherapy in early-stage disease, and avoiding anthracycline- or bleomycin-containing chemotherapy regimens. Evidence supporting the use of novel therapies remains limited with disappointing results from a recently published study of ibrutinib in patients with relapsed NLPHL. Hopefully, future trials will investigate novel agents such as checkpoint inhibitors, T-cell engaging antibodies and chimeric antigen receptor T-cell therapy.

中文翻译：

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结节性淋巴细胞为主的霍奇金淋巴瘤：疾病生物学、风险分层和治疗的进展。


最近的更新详细说明了结节性淋巴细胞为主的霍奇金淋巴瘤 （NLPHL） 患者如何使用预后评分系统更好地进行风险分层。大多数 NLPHL 患者表现为早期疾病，病程缓慢。为了反映与经典霍奇金淋巴瘤的这些差异，已经更新了命名法，以识别结节性淋巴细胞为主的 B 细胞淋巴瘤作为 NLPHL 的替代疗法。全球 NLPHL One 工作组于 2024 年发布了他们的关键数据集，该数据集挑战了变异免疫结构 （IAP） 模式的预后意义，并提出了一种新的预后评分系统。主要确定的预后因素包括年龄 >45 岁、III-IV 期疾病、血红蛋白 <10.5 g/dL 和脾受累。经过多变量分析，变异 IAP 未显示与较差结局相关。由于大多数 NLPHL 患者具有极好的长期生存率，因此确定适合治疗降级的患者将有助于最大限度地减少毒性。降级策略包括完全切除 I 期疾病后观察、主动监测、抗 CD20 抗体单药治疗、早期疾病放疗，以及避免使用含蒽环类药物或博来霉素的化疗方案。支持使用新疗法的证据仍然有限，最近发表的一项关于依鲁替尼治疗复发性 NLPHL 患者的研究结果令人失望。希望未来的试验将研究新型药物，例如检查点抑制剂、T 细胞结合抗体和嵌合抗原受体 T 细胞疗法。