Rosenwald Andreas, Menter Thomas, Dirnhofer Stefan
Institut für Pathologie, Universität Würzburg, Würzburg, Deutschland.
Pathologie, Institut für Medizinische Genetik und Pathologie, Universitätsspital Basel, Schönbeinstr. 40, 4031, Basel, Schweiz.
Pathologie (Heidelb). 2023 May;44(3):166-172. doi: 10.1007/s00292-023-01187-4. Epub 2023 Mar 14.
The 5th edition of the WHO classification (WHO-HAEM5) and the International Consensus Classification (ICC) show a broad consensus in the categorization of aggressive, large B‑cell lymphomas with expected minor impact only on the daily diagnostic routine. The changes compared to the 2017 revised WHO-HAEM4R are moderate and include updated names of entities, sharpened diagnostic criteria, and upgrades from provisional to definite entities. The definition of the most common aggressive B‑cell lymphoma, diffuse large B‑cell lymphoma (DLBCL), not otherwise specified (NOS), remains unchanged, and both classifications strongly encourage subtyping into germinal center B‑like (GCB) or the activated B‑like (ABC or non-GCB) DLBCL. DLBCL, NOS, should be separated from other large B‑cell lymphomas including large B‑cell lymphoma with IRF4 rearrangement (upgraded to a definite entity in both classifications) and large-cell/high-grade B‑cell lymphomas with 11q aberration. Aggressive B‑cell lymphomas with MYC and BCL2 rearrangements form a molecularly distinct group and are listed as definite entities in both classifications. This is in contrast to the more heterogeneous group of aggressive B‑cell lymphomas with MYC and BCL6 rearrangements that are recognized as a provisional entity in the ICC, while they fall into the DLBCL, NOS, or the HGBL, NOS, groups in the WHO-HAEM5.
世界卫生组织分类第5版(WHO-HAEM5)和国际共识分类(ICC)在侵袭性大B细胞淋巴瘤的分类方面显示出广泛的共识,预计对日常诊断流程的影响较小。与2017年修订的WHO-HAEM4R相比,变化较为温和,包括实体名称更新、诊断标准细化以及从临时实体升级为确定实体。最常见的侵袭性B细胞淋巴瘤——弥漫性大B细胞淋巴瘤(DLBCL),未另行规定(NOS)的定义保持不变,两种分类都强烈鼓励将其细分为生发中心B样(GCB)或活化B样(ABC或非GCB)DLBCL。NOS的DLBCL应与其他大B细胞淋巴瘤区分开来,包括伴有IRF4重排的大B细胞淋巴瘤(在两种分类中均升级为确定实体)以及伴有11q畸变的大细胞/高级别B细胞淋巴瘤。伴有MYC和BCL2重排的侵袭性B细胞淋巴瘤形成一个分子上独特的组,在两种分类中均列为确定实体。这与伴有MYC和BCL6重排的侵袭性B细胞淋巴瘤这一更为异质性的组形成对比,后者在ICC中被视为临时实体,而在WHO-HAEM5中则属于NOS的DLBCL或NOS的HGBL组。