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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.

DOI:10.1007/s00277-025-06330-0
PMID:40180648
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12141392/
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/effd6174c8cf/277_2025_6330_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b31/12141392/d2b8e16c372e/277_2025_6330_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b31/12141392/effd6174c8cf/277_2025_6330_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b31/12141392/d2b8e16c372e/277_2025_6330_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b31/12141392/effd6174c8cf/277_2025_6330_Fig2_HTML.jpg

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本文引用的文献

1
International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data.国际髓系肿瘤和急性白血病分类:整合形态学、临床和基因组数据。
Blood. 2022 Sep 15;140(11):1200-1228. doi: 10.1182/blood.2022015850.
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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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Molecular characterization and clinical outcome of B-cell precursor acute lymphoblastic leukemia with IG-MYC rearrangement.
IG-MYC 重排的 B 细胞前体急性淋巴细胞白血病的分子特征和临床结局。
Haematologica. 2023 Mar 1;108(3):717-731. doi: 10.3324/haematol.2021.280557.
4
Extranodal presentation of a lymphoma with precursor B-cell phenotype and translocation t(8;14) in South Africa.南非一例具有前体B细胞表型和t(8;14)易位的淋巴瘤的结外表现
Afr J Lab Med. 2022 Jan 31;11(1):1355. doi: 10.4102/ajlm.v11i1.1355. eCollection 2022.
5
Nationwide study of pediatric B-cell precursor acute lymphoblastic leukemia with chromosome 8q24/MYC rearrangement in Japan.日本全国范围内伴有染色体 8q24/MYC 重排的小儿 B 细胞前体急性淋巴细胞白血病的研究。
Pediatr Blood Cancer. 2020 Jul;67(7):e28341. doi: 10.1002/pbc.28341. Epub 2020 Apr 23.
6
Treatment and outcome of IG-MYC neoplasms with precursor B-cell phenotype in childhood and adolescence.儿童和青少年期具有前体B细胞表型的IG-MYC肿瘤的治疗与转归
Leukemia. 2020 Mar;34(3):942-946. doi: 10.1038/s41375-019-0606-6. Epub 2019 Oct 14.
7
IG- neoplasms with precursor B-cell phenotype are molecularly distinct from Burkitt lymphomas.具有前 B 细胞表型的 IG-肿瘤在分子上与伯基特淋巴瘤不同。
Blood. 2018 Nov 22;132(21):2280-2285. doi: 10.1182/blood-2018-03-842088. Epub 2018 Oct 3.
8
FAB LMB 96 Regimen for Newly Diagnosed Burkitt Lymphoma in Children: Single-center Experience.
J Pediatr Hematol Oncol. 2019 Jan;41(1):e7-e11. doi: 10.1097/MPH.0000000000001270.
9
The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.2016 年版世界卫生组织髓系肿瘤和急性白血病分类。
Blood. 2016 May 19;127(20):2391-405. doi: 10.1182/blood-2016-03-643544. Epub 2016 Apr 11.
10
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