Satou Akira, Takahara Taishi, Nakamura Shigeo
Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute 480-1195, Japan.
Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya 466-8550, Japan.
Cancers (Basel). 2022 May 26;14(11):2647. doi: 10.3390/cancers14112647.
Hodgkin lymphomas (HLs) are lymphoid neoplasms derived from B cells and consist histologically of large neoplastic cells known as Hodgkin and Reed-Sternberg cells and abundant reactive bystander cells. HLs include two main types, classic HL (CHL) and nodular lymphocyte predominant HL (NLPHL). Recent molecular analyses have revealed that an immune evasion mechanism, particularly the PD-1/PD-L1 pathway, plays a key role in the development of CHL. Other highlighted key pathways in CHL are NF-κB and JAK/STAT. These advances have dramatically changed the treatment for CHL, particularly relapsed/refractory CHL. For example, PD-1 inhibitors are now widely used in relapsed/refractory CHL. Compared with CHL, NLPHL is more characterized by preserved B cell features. Overlapping morphological and molecular features between NLPHL and T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) have been reported, and biological continuity between these two entities has been highlighted. Some THRLBCLs are considered to represent progression from NLPHLs. With considerable new understanding becoming available from molecular studies in HLs, therapies and classification of HLs are continually evolving. This paper offers a summary of and update on the pathological and molecular features of HLs for a better understanding of the diseases.
霍奇金淋巴瘤(HLs)是起源于B细胞的淋巴样肿瘤,组织学上由称为霍奇金和里德-斯腾伯格细胞的大肿瘤细胞以及丰富的反应性旁观者细胞组成。HLs包括两种主要类型,经典型HL(CHL)和结节性淋巴细胞为主型HL(NLPHL)。最近的分子分析表明,一种免疫逃逸机制,特别是PD-1/PD-L1途径,在CHL的发生发展中起关键作用。CHL中其他突出的关键途径是NF-κB和JAK/STAT。这些进展极大地改变了CHL的治疗方法,尤其是复发/难治性CHL的治疗。例如,PD-1抑制剂现在广泛用于复发/难治性CHL。与CHL相比,NLPHL更具有保留的B细胞特征。据报道,NLPHL与富含T细胞/组织细胞的大B细胞淋巴瘤(THRLBCL)之间存在重叠的形态学和分子特征,并且这两个实体之间的生物学连续性也得到了强调。一些THRLBCL被认为代表了NLPHL的进展。随着对HLs分子研究有了相当多的新认识,HLs的治疗方法和分类也在不断发展。本文对HLs的病理和分子特征进行了总结和更新,以便更好地了解这些疾病。