2022 年 WHO HAEM5 和国际淋巴瘤共识分类都改进了我们现在处理高级别 B 细胞淋巴瘤 (HGBL) 的方式，其中 MYC 和 BCL2 和/或 BCL6 重排使上一代分类向前迈进了一步。与形态相似但缺乏细胞遗传学异常分类的肿瘤相比，MYC/BCL2肿瘤的统一生物学更加清晰，其预后较差。 FISH 检测在很大程度上已成为基于人群的检测，我们从中学到了很多东西。我们可以轻松定义分子类别并将其广泛应用于临床实践。然而，MYC/BCL6 双易位在定义常见疾病组中的位置存在不确定性。我们增强了对结果和强化治疗在克服化疗耐药性方面的作用的了解。对于那些初始诱导化疗失败的患者，包括 CAR-T 疗法在内的免疫治疗方法正在改善预后。针对失调的致癌蛋白的新型抑制剂正在快速探索。罕见但困难的诊断分类 HGBL (NOS) 仍然是一种排除诊断，最佳临床方法的数据有限。宽松谈论双重和三重打击淋巴瘤的日子已经屈指可数了，因为这篇综述综合了 HGBL 中生物学、预后和治疗的当前数据。版权所有 © 2024 美国血液学会。

Both the 2022 WHO HAEM5 and the International Consensus Classification of lymphoma have refined the way we now approach high-grade B-cell lymphoma (HGBL) with MYC and BCL2 and/or BCL6 rearrangements moving the previous generation of classification a step forward. The unifying biology of MYC/BCL2 tumours has been clearer and their inferior prognosis confirmed compared to those with morphological similarities but lacking the classifying cytogenetic abnormalities. FISH testing has largely become population based and we have learnt much from this. We can readily define molecular categories and apply these widely to clinical practice. Uncertainty has however been shed upon the place of double MYC/BCL6 translocations in defining a common disease group. We have enhanced knowledge of outcomes and the role of therapy intensification to overcome chemotherapy resistance. For those patients failed by initial induction chemotherapy, immunotherapy approaches, including CAR-T therapies, are improving outcomes. Novel inhibitors, targeting dysregulated oncogenic proteins are being explored at pace. The rare, but difficult, diagnostic classification HGBL (NOS) remains a diagnosis of exclusion with limited data on an optimal clinical approach. The days of talking loosely of double and triple hit lymphoma are numbered as this review synergises the current data on biology, prognosis, and therapies in HGBL.Copyright © 2024 American Society of Hematology.