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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）没有显著差异。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

美国外科病理学杂志中文版2019年第二期摘要No.1

桑伟 翻译 张巍 审校

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