Haematology Department, Hospices Civils de Lyon, Lyon, France; University Lyon 1, Lyon, France.
Service d'Anatomie Pathologique, Hospices Civils de Lyon, Lyon, France; University Lyon 1, Lyon, France.
Lancet Oncol. 2015 Nov;16(15):e555-e567. doi: 10.1016/S1470-2045(15)00005-4.
Double-hit lymphoma (DHL) is a subgroup of aggressive lymphomas with both MYC and BCL2 gene rearrangements, characterised by a rapidly progressing clinical course that is refractory to aggressive treatment and short survival. Over time, the definition was modified and now includes diffuse large B-cell lymphoma (DLBCL) with MYC translocation combined with an additional translocation involving BCL2 or BCL6. Some cases that have a similar clinical course with concomitant overexpression of MYC or BCL2 proteins were recently characterised as immunohistochemical double-hit lymphomas (ie, double-protein-expression lymphomas [DPLs]). The clinical course of these DPLs is worse than so-called standard DLBCL but suggested by some studies to be slightly better than DHL, although there is overlap between the two categories. Present treatment does not allow cure or long-term survival in patients with genetic or immunohistochemical double-hit lymphomas, but several new drugs are being developed.
双打击淋巴瘤（DHL）是一种侵袭性淋巴瘤亚组，具有 MYC 和 BCL2 基因重排，其临床病程进展迅速，对侵袭性治疗和短期生存具有耐药性。随着时间的推移，其定义已被修改，现在包括 MYC 易位与另外涉及 BCL2 或 BCL6 的易位相结合的弥漫性大 B 细胞淋巴瘤（DLBCL）。最近，一些具有类似临床病程和 MYC 或 BCL2 蛋白过表达的病例被定义为免疫组织化学双打击淋巴瘤（即双蛋白表达淋巴瘤[DPL]）。这些 DPL 的临床病程比所谓的标准 DLBCL 更差，但一些研究表明比 DHL 略好，尽管这两个类别之间存在重叠。目前的治疗方法并不能治愈或长期生存具有遗传或免疫组织化学双打击淋巴瘤的患者，但正在开发几种新药。
