Borchmann Peter, Eichenauer Dennis A, Frigola Gerard, Campo Elias
First Department of Internal Medicine, Center for Integrated Oncology Aachen Bonn Cologne Dusseldorf, University of Cologne, Cologne, Germany.
German Hodgkin Study Group (GHSG), University Hospital Cologne, Cologne, Germany.
Hematol Oncol. 2025 Jun;43 Suppl 2(Suppl 2):e70080. doi: 10.1002/hon.70080.
Nodular lymphocyte-predominant B-cell/Hodgkin lymphoma (NLPB/HL) accounts for roughly 5% of all Hodgkin lymphoma (HL) cases. In contrast to classic HL (cHL), the malignant cells in NLPB/HL lack CD30 expression but maintain a complete phenotype of mature B-cells including CD20 and IgG, but occasionally show IgD expression. The tumor cells are known as lymphocyte-predominant (LP) cells and grow in a T-cell and histiocyte rich microenvironment against a background of follicular dendritic cells. Transformation to large B cell lymphoma may occur and requires excisional lymph node biopsies. Most patients with NLPB/HL present in early stages. The disease usually has an indolent clinical course. Approaches applied in cHL result in very good outcomes with only limited excess mortality in comparison with the general population. Activity has also been demonstrated for rituximab-containing regimens commonly used in indolent B-cell non-Hodgkin lymphoma. The present article reviews pathological characteristics, treatment options and tools that might improve risk stratification in NLPB/HL.
结节性淋巴细胞为主型B细胞/霍奇金淋巴瘤（NLPB/HL）约占所有霍奇金淋巴瘤（HL）病例的5%。与经典HL（cHL）不同，NLPB/HL中的恶性细胞缺乏CD30表达，但保留成熟B细胞的完整表型，包括CD20和IgG，但偶尔显示IgD表达。肿瘤细胞被称为淋巴细胞为主型（LP）细胞，在富含T细胞和组织细胞的微环境中生长，以滤泡树突状细胞为背景。可能会转化为大B细胞淋巴瘤，这需要切除性淋巴结活检。大多数NLPB/HL患者处于疾病早期。该疾病通常具有惰性临床病程。与普通人群相比，应用于cHL的治疗方法可产生非常好的效果，仅伴有有限的额外死亡率。含利妥昔单抗的方案在惰性B细胞非霍奇金淋巴瘤中常用，也已证明其有效性。本文综述了可能改善NLPB/HL风险分层的病理特征、治疗选择和工具。
