Section of Hematology/Oncology, Department of Medicine, University of Chicago, Chicago, Illinois.
Cancer. 2018 Dec 15;124(24):4622-4632. doi: 10.1002/cncr.31646. Epub 2018 Sep 25.
Emerging biologic subsets and new prognostic markers are significantly and adversely affecting curability after standard chemoimmunotherapy for aggressive B-cell lymphomas. The identification of concurrent MYC and B-cell CLL/lymphoma 2 (BCL2) deregulation, whether at a genomic or protein level, has opened a new era of investigation within the most common subtype of aggressive B-cell lymphomas. Double-hit lymphoma (DHL), defined as a dual rearrangement of MYC and BCL2 and/or B-cell CLL/lymphoma 6 (BCL6) genes, is an uncommon subset accounting for 5% to 7% of all diffuse large B-cell lymphomas (DLBCLs), and long-term survivors are rare. Double-expressor lymphoma (DEL), defined as overexpression of MYC and BCL2 proteins not related to underlying chromosomal rearrangements, is not a distinct entity in the current World Health Organization classification but accounts for 20% to 30% of DLBCL cases and also has poor outcomes. There are many practical considerations related to identifying, determining the prognosis of, and managing DHL and DEL.
在标准的化疗免疫治疗后，侵袭性 B 细胞淋巴瘤的治愈率因新兴的生物学亚型和新的预后标志物而显著降低，预后不良。无论是在基因组水平还是蛋白水平上，同时检测到 MYC 和 B 细胞慢性淋巴细胞白血病/淋巴瘤 2（BCL2）的失调，已经为最常见的侵袭性 B 细胞淋巴瘤亚型开辟了一个新的研究领域。双打击淋巴瘤（DHL）定义为 MYC 和 BCL2 及/或 B 细胞慢性淋巴细胞白血病/淋巴瘤 6（BCL6）基因的双重重排，占所有弥漫性大 B 细胞淋巴瘤（DLBCL）的 5%至 7%，长期生存者罕见。双表达淋巴瘤（DEL）定义为 MYC 和 BCL2 蛋白的过度表达，与潜在的染色体重排无关，在目前的世界卫生组织分类中不是一个独立的实体，但占 DLBCL 病例的 20%至 30%，预后也较差。在识别、确定 DHL 和 DEL 的预后以及管理方面有许多实际的考虑因素。
