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弥漫性大 B 细胞淋巴瘤 1.2016 年版

Diffuse Large B-Cell Lymphoma Version 1.2016.

出版信息

J Natl Compr Canc Netw. 2016 Feb;14(2):196-231. doi: 10.6004/jnccn.2016.0023.

DOI:10.6004/jnccn.2016.0023
PMID:26850490
Abstract

Diffuse large B-cell lymphomas (DLBCL) are now considered a heterogeneous group of distinct molecular subtypes (germinal center B-cell DLBCL, activated B-cell DLBCL, and primary mediastinal large B-cell lymphoma (PMBL) with varied natural history and response to therapy. In addition, a subset of patients with DLBCL have concurrent MYC and/or BCL2 gene rearrangements (double-hit lymphomas; DHL) and others have a dual expression of both MYC and BCL2 proteins (double-expressing DLBCL; DEL). The standard of care for the treatment of patients with PMBL, DHL, or DEL has not been established. Adequate immunophenotyping and molecular testing (in selected circumstances) are necessary for the accurate diagnosis of different subtypes of DLBCL. The NCCN Guidelines included in this issue, part of the NCCN Guidelines for non-Hodgkin's lymphomas, address the diagnosis and management of DLBCL and its subtypes.

摘要

弥漫性大 B 细胞淋巴瘤 (DLBCL) 现在被认为是一组具有不同分子亚型的异质性疾病(生发中心 B 细胞型 DLBCL、活化 B 细胞型 DLBCL 和原发性纵隔大 B 细胞淋巴瘤 (PMBL),它们具有不同的自然病史和对治疗的反应。此外,一部分 DLBCL 患者存在同时发生的 MYC 和/或 BCL2 基因重排(双打击淋巴瘤;DHL),而另一些患者则同时表达 MYC 和 BCL2 蛋白(双表达 DLBCL;DEL)。PMBL、DHL 或 DEL 患者的标准治疗尚未建立。为了准确诊断不同类型的 DLBCL,需要进行充分的免疫表型和分子检测(在特定情况下)。本期 NCCN 指南是 NCCN 非霍奇金淋巴瘤指南的一部分,涵盖了 DLBCL 及其亚型的诊断和管理。

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