Department of Haematology, Monash Health, Clayton, Victoria, Australia; School of Clinical Sciences at Monash Health, Monash University, Clayton, Victoria.
Department of Anatomical Pathology, PathWest, Nedlands, Western Australia.
Haematologica. 2024 Nov 1;109(11):3476-3487. doi: 10.3324/haematol.2024.285903.
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.
最近的更新详细说明了如何使用预后评分系统更好地对结节性淋巴细胞为主型霍奇金淋巴瘤 (NLPHL) 患者进行风险分层。大多数 NLPHL 患者表现为早期疾病，且疾病呈惰性过程。为了反映与经典霍奇金淋巴瘤的这些差异，命名法已更新，将结节性淋巴细胞为主型 B 细胞淋巴瘤作为 NLPHL 的替代物加以识别。全球 NLPHL 工作组于 2024 年公布了其关键数据集，该数据集对变异免疫结构 (IAP) 模式的预后意义提出了挑战，并提出了一种新的预后评分系统。关键的预后因素包括年龄>45 岁、III-IV 期疾病、血红蛋白<10.5 g/dL 和脾脏受累。多变量分析后，变异 IAP 与不良结局无关。由于大多数 NLPHL 患者具有极好的长期生存，因此确定适当的治疗降级患者将有助于最大限度地降低毒性。降级策略包括完全切除 I 期疾病后的观察、主动监测、抗 CD20 抗体单药治疗、早期疾病的放疗以及避免使用含蒽环类或博来霉素的化疗方案。支持使用新型疗法的证据仍然有限，最近发表的一项关于复发型 NLPHL 患者使用伊布替尼的研究结果令人失望。希望未来的试验将研究新型药物，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。
