Nodular lymphocyte predominant Hodgkin lymphoma: pathology, clinical course and relation to T-cell/histiocyte rich large B-cell lymphoma
Nodular lymphocyte predominant Hodgkin lymphoma: pathology, clinical course and relation to T-cell/histiocyte rich large B-cell lymphoma
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DOI：
10.1016/j.pathol.2019.10.003
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发表时间：
2020-01-01
期刊：
影响因子：
4.5
通讯作者：
Eichenauer, Dennis A.
中科院分区：
文献类型：
--
作者：
Hartmann, Sylvia;Eichenauer, Dennis A.
结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）是一种罕见的霍奇金淋巴瘤亚型，具有独特的组织病理学和临床表现。它主要影响男性，在大多数情况下表现为局部疾病和无痛临床过程。然而，也有晚期NLPHL患者经常出现脾脏和肝脏受累，B症状和更具侵略性的临床过程。不同的临床表现与不同的组织病理学特征相关。NLPHL可分为典型的和变异的组织病理学生长模式。具有典型生长模式的大多数患者的临床过程是惰性的，而具有变异组织学的患者更常表现为晚期疾病，并且复发发生更频繁和更早。尽管存在这些差异，但阶段适应性治疗后的预后对两组患者都有利。一些具有不同组织学表现的病例显示与富含T细胞/组织细胞的大B细胞淋巴瘤（THRLBCL）的组织病理学和临床重叠。虽然被认为是侵袭性B细胞淋巴瘤，THRLBCL表现出许多类似于NLPHL的特征，表明在发病机制方面的密切关系。这两种淋巴瘤实体都来源于生发中心B细胞，显示出持续的体细胞超突变，并且在肿瘤细胞的基因表达、基因组失衡和突变模式方面彼此相似。
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an unusual subtype of Hodgkin lymphoma characterised by a distinct histopathological and clinical presentation. It mostly affects males and presents with localised disease and an indolent clinical course in the majority of cases. However, there are also patients with advanced NLPHL who frequently present with spleen and liver involvement, B-symptoms and a more aggressive clinical course. Different clinical presentations correlate with distinct histopathological characteristics. NLPHL can be divided into typical and variant histopathological growth patterns. The clinical course of most patients with a typical growth pattern is indolent whereas patients with a variant histology more often present with advanced stage disease and relapse occurs more frequently and earlier. Despite these differences, the prognosis after stage-adapted treatment is favourable for both patient groups. Some cases presenting with a variant histology show a histopathological and clinical overlap with T-cell/histiocyte rich large B-cell lymphoma (THRLBCL). Although being considered as aggressive B-cell lymphoma, THRLBCL exhibits many features that are similar to NLPHL, indicating a close relationship with regard to pathogenesis. Both lymphoma entities derive from germinal centre B-cells, show ongoing somatic hypermutation, and resemble each other in terms of gene expression of tumour cells, genomic imbalances and mutation patterns.