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复发或进展后的边缘区黏膜相关淋巴组织淋巴瘤患者中异常的bcl6蛋白表达:两例病例报告

Aberrant bcl6 protein expression in patients with EMZL following recurrence or progression: case report of two cases.

作者信息

Zhang Ming, Qu Jianli, Guo Weiwei, Liu Yi, Zhang Wei

机构信息

Department of Pathology, Yantaishan Hospital Affiliated to Binzhou Medical University, Yantai, 264003, Shandong, China.

Department of Hematology, Yantaishan Hospital Affiliated to Binzhou Medical University, Yantai, 264003, Shandong, China.

出版信息

Diagn Pathol. 2024 Dec 26;19(1):165. doi: 10.1186/s13000-024-01593-z.

DOI:10.1186/s13000-024-01593-z
PMID:39726003
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11670503/
Abstract

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (EMZL) is an indolent B-cell lymphoma that can involve various anatomic sites. EMZL is derived from post-germinal center marginal zone B cells and typically lacks bcl-6 expression. Herein, we report two post-treatment cases of EMZL where unexpected bcl-6 protein expression was observed in specimens obtained following recurrence or progression. This contrasts with the primary specimens, which were negative for the bcl-6. Additionally, we confirm that the altered bcl6 expression observed in relapsed EMZL cases is independent of BCL6 gene rearrangement, as demonstrated by fluorescence in situ hybridization analysis. Relevant literature was reviewed and summarized to enhance the understanding of this phenomenon, particularly for pathologists.

摘要

黏膜相关淋巴组织结外边缘区淋巴瘤(EMZL)是一种惰性B细胞淋巴瘤,可累及多个解剖部位。EMZL起源于生发中心后边缘区B细胞,通常缺乏bcl-6表达。在此,我们报告2例EMZL治疗后病例,在复发或进展后获取的标本中观察到意外的bcl-6蛋白表达。这与原发性标本形成对比,原发性标本的bcl-6为阴性。此外,我们证实复发EMZL病例中观察到的bcl6表达改变与BCL6基因重排无关,荧光原位杂交分析已证明这一点。我们查阅并总结了相关文献,以增进对这一现象的理解,特别是对病理学家而言。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/d2c6172d207a/13000_2024_1593_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/ea7b1a08c080/13000_2024_1593_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/dbeb7ad47724/13000_2024_1593_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/996e6fdb7b90/13000_2024_1593_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/d2c6172d207a/13000_2024_1593_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/ea7b1a08c080/13000_2024_1593_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/dbeb7ad47724/13000_2024_1593_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/996e6fdb7b90/13000_2024_1593_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f7f/11670503/d2c6172d207a/13000_2024_1593_Fig4_HTML.jpg

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本文引用的文献

1
The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms.《世界卫生组织造血与淋巴组织肿瘤分类》第五版:淋巴肿瘤。
Leukemia. 2022 Jul;36(7):1720-1748. doi: 10.1038/s41375-022-01620-2. Epub 2022 Jun 22.
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The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee.成熟淋巴细胞肿瘤国际共识分类:临床咨询委员会报告。
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Expression of germinal center cell markers by extranodal marginal zone lymphomas of MALT type within colonized follicles, a diagnostic pitfall with follicular lymphoma.
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Leuk Lymphoma. 2021 May;62(5):1116-1122. doi: 10.1080/10428194.2020.1855347. Epub 2020 Dec 7.
4
Reciprocal negative regulation between the tumor suppressor protein p53 and B cell CLL/lymphoma 6 (BCL6) via control of caspase-1 expression.肿瘤抑制蛋白 p53 通过控制半胱氨酸蛋白酶-1 的表达与 B 细胞慢性淋巴细胞白血病/淋巴瘤 6(BCL6)之间的相互负调控。
J Biol Chem. 2019 Jan 4;294(1):299-313. doi: 10.1074/jbc.RA118.004204. Epub 2018 Nov 8.
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Inhibition of intracellular lipolysis promotes human cancer cell adaptation to hypoxia.抑制细胞内脂肪分解促进人类癌细胞适应缺氧。
Elife. 2017 Dec 19;6:e31132. doi: 10.7554/eLife.31132.
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Germinal center B cell development has distinctly regulated stages completed by disengagement from T cell help.生发中心B细胞的发育具有明显受调控的阶段,这些阶段通过与T细胞辅助脱离而完成。
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7
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Blood. 2006 Oct 1;108(7):2373-83. doi: 10.1182/blood-2006-05-022517. Epub 2006 Jun 13.