进一步探究伴有MYC基因异常的大B细胞淋巴瘤的复杂性以及母细胞样形态的重要性
  ——本文经《美国外科病理学杂志》授权发布,其他媒体转载或引用须经《美国外科病理学杂志》同意,否则追究法律责任。
 
  2016年世界卫生组织分类认知到“伴有MYC和BCL2和/或BCL6基因重排的高级别B细胞淋巴瘤”[双重/三重打击淋巴瘤(double/triple-hit lymphoma,DTHL)]和“非特指性高级别B细胞淋巴瘤”这两类肿瘤,后者包括伴有“母细胞样”或者“中间”细胞学形态的非DTHL病例。尽管曾被广泛研究,仍存在许多问题,包括:哪些病例应该归入这些类别?哪些因素能减轻它们的不利预后?以及何时去做荧光原位杂交研究?因此,我们通过MYC、BCL2和BCL6荧光原位杂交研究了187例大B细胞淋巴瘤的临床病理特征。其中,有47例DTHL病例、36例伴有MYC和BCL2和/或BCL6额外信号(extra signals,ES)和/或基因重排的病例(ES组,排除DTHL病例)、9例仅伴有MYC重排(单一打击淋巴瘤)以及95例不伴MYC异常(abnormalities,NM)的病例。与不伴MYC异常患者相比,罹患DTHL而非单一打击淋巴瘤患者的预后更差(P=0.0079)。与NM/无重排ES组患者相比,伴有至少一个基因重排的ES组患者预后更差(P<0.02)。DTHL多为母细胞样而非中间细胞学形态的病例(P<0.0001),且母细胞样者预后显著较差,后者即便在DTHL病例中亦是如此(P=0.0282)。弥漫性大B细胞淋巴瘤和中间形态组的预后相似。无论针对全组病例还是DTHL病例,国际预后指数评分都对预后有着重要意义(P=0.0074)。约93%的DTHL病例呈GCB亚型,但24%的DTHL病例中MYC阳性细胞低于40%。在DTHL病例中,MYC+BCL2+双重表达的病例预后较差(P=0.0328)。这些结果突显了形态、表型以及临床变异在DTHL中的重要性,并提示,不能仅根据MYC、BCL2和/或BCL6基因的额外信号(ES)作出DTHL的诊断。
  来源:Am J Surg Pathol 2017;41:1155–1166
  美国外科病理学杂志中文版2018年第一期摘要No.3
  (李小秋 翻译/审校)
 
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