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伴 MYC、BCL2 和 BCL6 重排的 TdT 阳性淋巴瘤:诊断与治疗综述

英文原题:TdT Positive Lymphoma with MYC, BCL2 and BCL6 Rearrangements: A Review of Diagnosis and Treatment.

查看英文原题

TdT Positive Lymphoma with MYC, BCL2 and BCL6 Rearrangements: A Review of Diagnosis and Treatment.

PubMed 2022/03/01(内容时间) Mediterr J Hematol Infect Dis Q3 · IF 2.3(JCR 2025)

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中文摘要

现代肿瘤分类不仅依据免疫形态学特征,还取决于特定的疾病定义性遗传事件。MYC/8q24位点易位或重排合并BCL2或BCL6易位(双打击/三打击),被视为侵袭性成熟B细胞淋巴瘤——高级别B细胞淋巴瘤(HGBL)的标志。当具有未成熟免疫表型的病例出现这些重排时,诊断会非常困难。本文报告一例罕见的侵袭性B细胞淋巴瘤/白血病,具有未成熟形态和免疫表型,同时伴有三打击基因重排。该病例凸显了此类患者分类和规范治疗的困难。其新颖之处在于患者接受了嵌合抗原受体(CAR)T细胞治疗,并报告了治疗结局。

展开英文摘要原文

In the modern era, classification of neoplasms not only depends on immunomorphological features but also on specific disease-defining genetic events. Translocations/rearrangements of MYC/8q24 locus combined with BCL-2 or BCL6 translocations (double/triple hit) are considered hallmarks of high-grade B-cell lymphoma (HGBL), a type of aggressive mature B-cell lymphoma. When cases with immature immunophenotypes present these rearrangements, diagnosis becomes very difficult.

We herein report an unusual case of an aggressive B-cell lymphoma/leukemia that presented with immature morphology and immunophenotype with triple hit gene rearrangements. This case highlights the difficulty in classifying and appropriately treating these patients. The novel aspect is the treatment and outcome with chimeric antigen receptor or CAR T -cell therapy .

论文信息

作者
Singh A、Asghar I、Kohler L、Snower D、Hakim H、Lebovic D
单位
Department of Hematology and Oncology, Ascension St. John Hospital.
文献类型
病例报告
期刊
Mediterranean journal of hematology and infectious diseases2022
原文标识
PubMed 35444774 · DOI 10.4084/MJHID.2022.018