Nodular lymphocyte-predominant Hodgkin lymphoma: advances in disease biology, risk stratification, and treatment.

IF 8.2 1区 医学 Q1 HEMATOLOGY
Ross T Salvaris, Benjamin M Allanson, Graham Collins, Chan Cheah
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引用次数: 0

Abstract

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.

结节性淋巴细胞为主的霍奇金淋巴瘤:疾病生物学、风险分层和治疗方面的进展。
最近的更新详细介绍了如何利用预后评分系统更好地对结节性淋巴细胞占优势的霍奇金淋巴瘤(NLPHL)患者进行风险分层。大多数 NLPHL 患者都是早期发病,病程缓慢。为了反映这些与典型霍奇金淋巴瘤的不同之处,对术语进行了更新,将结节性淋巴细胞占优势的B细胞淋巴瘤作为NLPHL的替代。全球NLPHL工作组于2024年公布了他们的关键数据集,该数据集对变异免疫结构(IAP)模式的预后意义提出了质疑,并提出了新的预后评分系统。已确定的主要预后因素包括年龄大于 45 岁、III-IV 期疾病、血红蛋白。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Haematologica
Haematologica 医学-血液学
CiteScore
14.10
自引率
2.00%
发文量
349
审稿时长
3-6 weeks
期刊介绍: Haematologica is a journal that publishes articles within the broad field of hematology. It reports on novel findings in basic, clinical, and translational research. Scope: The scope of the journal includes reporting novel research results that: Have a significant impact on understanding normal hematology or the development of hematological diseases. Are likely to bring important changes to the diagnosis or treatment of hematological diseases.
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