Abstract：
A small subset of high-grade B-cell lymphoma (HGBL) with blastoid morphology remains poorly understood. We assessed 55 cases of blastoid HGBL, not otherwise specified (NOS) and compared their clinicopathologic characteristics with those of 81 non-blastoid HGBL-NOS and 62 blastoid HGBL with MYC and BCL2, with or without BCL6 rearrangements (double/triple-hit lymphoma [D/THL]). Patients with blastoid HGBL-NOS showed similar clinicopathologic features to patients with blastoid D/THLs and non-blastoid HGBL-NOS, except more frequently with a history of low-grade B-cell lymphoma, bone marrow involvement, and BCL2 rearrangement (P < .05) compared to the latter. MYC rearrangement (MYC-R), detected in 40% of blastoid HGBL-NOS, was associated with aggressive clinicopathologic features and poorer overall survival, even worse than that of blastoid D/THL (P < .05). Transcriptome profiling revealed a distinct gene expression pattern with differentially expressed genes enriched in MYC and P53-targeted genes in MYC-R blastoid HGBL-NOS. Fifty-two percent of blastoid HGBL-NOS had a double hit-like signature, similar to non-blastoid HGBL-NOS (P = .73). The overall survival of the blastoid HGBL-NOS group was similar to that of the blastoid D/THL group but appeared poorer than that of its non-blastoid counterparts (P = .07). Taken together, blastoid HGBL-NOS is an aggressive B-cell lymphoma that shares overlapping clinicopathologic and genetic features with non-blastoid HGBL-NOS. MYC-R in patients with blastoid HGBL-NOS identifies a highly aggressive subgroup with distinct aggressive clinicopathologic features, unique molecular signatures, and a dismal clinical outcome.
摘要：
一小部分具有囊样形态的高级B细胞淋巴瘤（HGBL）仍然知之甚少。我们评估了55例囊状HGBL-NOS，并将其临床病理特征与81例非囊状HGBL-NOS和62例有MYC的囊状HGBL进行了比较。BCL2,有/无BCL6重排(D/THL)。囊样HGBL-NOS患者的临床病理特征与囊样D/THL和非囊样HGBL-NOS患者相似，除了经常有低度B细胞淋巴瘤的病史，骨髓受累与BCL2-R(p<0.05)比拟后者。MYC重排(MYC-R),在40%的囊样HGBL-NOS中检测到，与侵袭性临床病理特征和较差的总生存率（OS）相关，甚至比囊样D/THL更差(p<0.05)。转录组谱分析揭示了一种独特的基因表达模式，在MYC-R囊样HGBL-NOS中富含MYC和P53靶向基因的差异表达基因。52%的囊样HGBL-NOS具有DH样特征，与非囊样HGBL-NOS相似(p=0.73)。囊样HGBL-NOS组的OS与囊样D/THL组相似，但比非囊样组较差（p=0.07）。一起来看,囊样HGBL-NOS是一种侵袭性B细胞淋巴瘤，与非囊样HGBL-NOS具有重叠的临床病理和遗传特征。囊样HGBL-NOS患者的MYC-R确定了一个具有明显侵袭性临床病理特征的高度侵袭性亚组。独特的分子特征和令人沮丧的临床结果。
