Fakhruddin Najla, Abou Dalle Iman, Chakhachiro Zaher
American University of Beirut, Beirut, Lebanon.
American University of Beirut, Beirut, Lebanon.
Hum Pathol. 2025 Feb;156:105705. doi: 10.1016/j.humpath.2024.105705. Epub 2024 Dec 10.
Primary mediastinal large B-cell lymphoma (PMBL) is a mature aggressive B-cell lymphoma that arises in the anterior mediastinum, likely originating from thymic B cells. Initially considered a subtype of diffuse large B-cell lymphoma, PMBL has since been established as a distinct clinicopathologic entity due to its unique clinical, morphologic, immunophenotypic and genetic characteristics. PMBL primarily affects young adults, especially women, and manifests as a bulky mediastinal mass that can invade adjacent structures, often causing respiratory symptoms. The genomic landscape of PMBL includes alterations in the JAK-STAT, NF-κB signaling pathways, and immune evasion mechanisms. This review explores the clinical presentation, pathogenesis and genetic landscape of PMBL, highlighting its morphologic and immunophenotypic characteristics and differences from related mediastinal lymphomas such as classic Hodgkin lymphoma and mediastinal grey zone lymphoma. We also discuss the implications of these findings on diagnosis, management and personalized treatment approaches.
原发性纵隔大B细胞淋巴瘤（PMBL）是一种成熟的侵袭性B细胞淋巴瘤，起源于前纵隔，可能源自胸腺B细胞。PMBL最初被认为是弥漫性大B细胞淋巴瘤的一种亚型，由于其独特的临床、形态、免疫表型和基因特征，现已被确立为一种独特的临床病理实体。PMBL主要影响年轻成年人，尤其是女性，表现为巨大的纵隔肿块，可侵犯相邻结构，常导致呼吸道症状。PMBL的基因组图谱包括JAK-STAT、NF-κB信号通路的改变以及免疫逃逸机制。本综述探讨了PMBL的临床表现、发病机制和基因图谱，重点介绍了其形态和免疫表型特征以及与相关纵隔淋巴瘤（如经典霍奇金淋巴瘤和纵隔灰色地带淋巴瘤）的差异。我们还讨论了这些发现对诊断、管理和个性化治疗方法的影响。
