Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, 77030, USA.
Curr Hematol Malig Rep. 2013 Sep;8(3):243-52. doi: 10.1007/s11899-013-0169-y.
Diffuse large B-cell lymphoma (DLBCL) is heterogeneous. Gene expression profiling (GEP) has identified two principal subtypes: germinal center B cell (GCB) and activated B cell (ABC). Most DLBCL cases are distinct from Burkitt lymphoma (BL), but a subset of tumors has a GEP profile between BL and DLBCL, suggesting a spectrum. In parallel, the 2008 World Health Organization (WHO) classification included the category of B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL (BCL-U). MYC rearrangement and potential synergy with other genetic aberrations, particularly BCL2 or BCL6, so-called double hit lymphoma, have also been studied in DLBCL and gray zone lymphoma. These subsets have been associated with a poor patient outcome, with the data being strongest for MYC/BCL2 double hit lymphomas. This review summarizes the literature on the impact of MYC rearrangement, as well as MYC/BCL2 double hit, in patients with DLBCL and BCL-U. We also emphasize the evolving nature of these concepts, and outline suggestions for future studies.
弥漫性大 B 细胞淋巴瘤 (DLBCL) 具有异质性。基因表达谱 (GEP) 已确定了两种主要亚型：生发中心 B 细胞 (GCB) 和活化 B 细胞 (ABC)。大多数 DLBCL 病例与伯基特淋巴瘤 (BL) 不同，但肿瘤的一部分具有 BL 和 DLBCL 之间的 GEP 特征，提示存在一个谱系。与此同时，2008 年世界卫生组织 (WHO) 分类包括了介于 DLBCL 和 BL 之间的 B 细胞淋巴瘤，不可分类，具有介于两者之间的特征 (BCL-U)。MYC 重排以及与其他遗传异常的潜在协同作用，特别是 BCL2 或 BCL6，即所谓的双重打击淋巴瘤，也在 DLBCL 和灰色区域淋巴瘤中进行了研究。这些亚组与患者预后不良相关，MYC/BCL2 双重打击淋巴瘤的数据最强。这篇综述总结了关于 MYC 重排在 DLBCL 和 BCL-U 患者中的影响，以及 MYC/BCL2 双重打击的文献。我们还强调了这些概念的不断发展性质，并概述了未来研究的建议。
