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儿童伴有C-MYC重排的B淋巴细胞淋巴瘤病例系列:诊断和治疗挑战

A case series of B-Lymphoblastic lymphoma with C-MYC rearrangement in children: diagnostic and therapeutic challenges.

作者信息

Yang Xueliang, Duan Yanlong, Zhou Chunju, Zhang Ningning, Bai Xueqing, Jin Ling, Huang Shuang, Zhang Meng

机构信息

Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

National Key Clinical Discipline of Pediatric Hematology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing, China.

出版信息

Ann Hematol. 2025 May;104(5):3059-3066. doi: 10.1007/s00277-025-06330-0. Epub 2025 Apr 4.

Abstract

C-MYC rearrangement has been listed as a new subcategory of B-cell acute lymphoblastic leukemia (B-ALL) in the latest International Consensus Classification (ICC), but B-cell lymphoblastic lymphoma (B-LBL) with C-MYC rearrangement is extremely rare and therefore is a novel finding worth reporting. In this study, we described a small number of pediatric cases of B-LBL with C-MYC rearrangement, which overlapped features of B-LBL and Burkitt lymphoma (BL), including highly aggressive clinical presentation, lymphoblasts with precursor B-cell phenotype and C-MYC rearrangement; the combination treatment of the mature B-NHL protocol and ALL-type protocol may be appropriate for this rare entity, and more studies are required to identify an adequate therapeutic strategy.

摘要

C-MYC重排在最新的国际共识分类(ICC)中已被列为B细胞急性淋巴细胞白血病(B-ALL)的一个新亚类,但伴有C-MYC重排的B细胞淋巴母细胞淋巴瘤(B-LBL)极为罕见,因此是一个值得报告的新发现。在本研究中,我们描述了少数伴有C-MYC重排的儿童B-LBL病例,其具有B-LBL和伯基特淋巴瘤(BL)的重叠特征,包括高度侵袭性的临床表现、具有前体B细胞表型的淋巴母细胞和C-MYC重排;成熟B-NHL方案和ALL型方案的联合治疗可能适用于这种罕见疾病,还需要更多研究来确定合适的治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b31/12141392/d2b8e16c372e/277_2025_6330_Fig1_HTML.jpg

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