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伯基特淋巴瘤与MYC:还有什么新情况?

Burkitt lymphoma and MYC: what else is new?

作者信息

Said Jonathan, Lones Mark, Yea Steven

机构信息

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, UCLA, Los Angeles, CA.

出版信息

Adv Anat Pathol. 2014 May;21(3):160-5. doi: 10.1097/PAP.0b013e3182a92cde.

Abstract

Burkitt lymphoma (BL) is the most common non-Hodgkin lymphoma in children and adolescents, but at least 30% of cases occur in patients older than 60 years, and the absolute number of BL cases in adults exceeds those in childhood. BL is described as a monomorphic proliferation of medium-sized transformed B cells with round nuclei, clumped chromatin, basophilic cytoplasm, and squared-off cell borders, cytoplasmic vacuoles, medium-sized paracentral nucleoli, and a starry sky pattern. Translocation involving MYC is characteristic but not specific for BL. No single parameter is the gold standard for diagnosis; morphology, cytogenetics, immunophenotype, and gene expression profiles all may contribute to the diagnosis. Although neither EBV nor MYC are sufficient to cause BL there is increasing information from techniques such as complete RNA sequencing that identify essential pathways that are activated in the pathogenesis of BL. These findings suggest novel opportunities for improved therapeutic intervention.

摘要

伯基特淋巴瘤(BL)是儿童和青少年中最常见的非霍奇金淋巴瘤,但至少30%的病例发生在60岁以上的患者中,并且成人BL病例的绝对数量超过儿童。BL被描述为中等大小转化B细胞的单形性增殖,这些细胞具有圆形核、块状染色质、嗜碱性细胞质、方形细胞边界、细胞质空泡、中等大小的核旁核仁以及满天星图案。涉及MYC的易位是BL的特征性表现,但并非特异性表现。没有单一参数是诊断的金标准;形态学、细胞遗传学、免疫表型和基因表达谱都可能有助于诊断。尽管EBV和MYC都不足以导致BL,但来自全RNA测序等技术的信息越来越多,这些技术确定了在BL发病机制中被激活的关键途径。这些发现为改进治疗干预提供了新的机会。

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