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  原发性皮肤弥漫大B细胞淋巴瘤,腿型(primary cutaneous diffuse large B-cell lymphoma, leg type, PCDLBCL-LT)和原发性皮肤滤泡中心淋巴瘤(primary cutaneous follicle center lymphoma, PCFCL)是具不同临床特征和生物学行为的皮肤B细胞淋巴瘤(cutaneous B-cell lymphoma, CBCL)。PCDLBCL-LT是侵袭性最强的CBCL,预后相对较差。在结内弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma, DLBCL)中,MYC重排,特别是伴有BCL2和/或BCL6二次打击,以及MYC和BCL2的双蛋白表达(double protein expression,DE)是不利的预后因素。由于这些因素在CBCL中的临床意义尚不清楚,我们在44例PCDLBCL-LT患者和17例PCFCL患者中研究了MYC重排和DE的发生率及预后价值。与结内DLBCL(9%-14%)相比,PCDLBCL-LT患者MYC重排发生率(32%)较高,但仅有2例(4%)发生二次打击,均为BCL6。具有MYC重排的PCDLBCL-LT患者显示较短的疾病特异性生存期(对数秩,P=0.036)和无病生存期(对数秩,P=0.028),但与无MYC重排相比,5年总生存期(对数秩,P=0.157)没有显着差异。DE在PCDLBCL-LT患者?中的发生率为65%,与生产率降低无相关性。PCFCL组未见MYC重排和DE。总之,本研究发现,与结内DLBCL相比,PCDLBCL-LT的MYC重排发生率较高,且进一步表明,MYC重排是预后较差的指标。因此,我们的数据表明,对所有新诊断的PCDLBCL-LT病例进行MYC-FISH检测是有意义的。
Am J Surg Pathol 2018;42:1488-1494
桑伟 翻译 张巍 审校
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