滤泡性淋巴瘤和弥漫性大B细胞淋巴瘤定义的演变:个性化医学的未来模型

IF 0.9 Q4 HEMATOLOGY Hemato Pub Date : 2022-07-21 DOI:10.3390/hemato3030032
E. Jaffe, A. Carbone
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引用次数: 2

摘要

滤泡性淋巴瘤(FL)和弥漫性大B细胞淋巴瘤(DLBCL)的定义在个性化医学时代不断发展。FL进化的早期阶段已经得到了认可。早期病变的两种组织学表现是原位卵泡瘤变和十二指肠型FL。此外,FL经常发生组织学转变,最常见的形式是DLBCL。高级别B细胞淋巴瘤伴双重侵袭,易位涉及BCL2和MYC,在临床上具有重要意义。罕见的转化形式包括典型的霍奇金淋巴瘤(CHL)和组织细胞肉瘤。除了与BCL2易位相关的传统FL外,还观察到了BCL2阴性FL的替代形式。这些在临床和基因上都是异质性的。毛囊细胞衍生的一组独特的B细胞淋巴瘤发生在年轻患者中,包括儿童FL型、睾丸FL和一种伴有IRF4重排的大B细胞淋巴瘤。从历史上看,DLBCL只分为两种组织学变体,中心母细胞和免疫母细胞。2017年,世界卫生组织分类建议(1)分离活化的B细胞和生发中心B细胞衍生的DLBCL,(2)识别具有双重打击的高级B细胞淋巴瘤,以及(3)识别可能类似Burkitt淋巴瘤的侵袭性淋巴瘤,目前在国际共识分类中被指定为具有11q畸变的大B细胞淋巴瘤。今天,我们认识到侵袭性B细胞淋巴瘤的基因组复杂性更高。最近对NGS和突变谱的研究已经确定了具有临床意义的遗传亚群。希望这些数据最终将导致基于基因图谱的靶向治疗。
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Evolution in the Definition of Follicular Lymphoma and Diffuse Large B-Cell Lymphoma: A Model for the Future of Personalized Medicine
The definitions of follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) are evolving in the era of personalized medicine. Early stages of the evolution of FL have been recognized. Two histological manifestations of early lesions are in situ follicular neoplasia and duodenal type FL. Additionally, FL frequently undergoes histological transformation, the most common form being DLBCL. High-grade B-cell lymphoma with double hit, with translocations involving BCL2 and MYC are important clinically. Rarer forms of transformation include classic Hodgkin lymphoma (CHL) and histiocytic sarcoma. In addition to conventional FL associated with the BCL2 translocation, alternative forms of BCL2-negative FL have been observed. These are heterogenous clinically and genetically. A distinctive group of B-cell lymphomas of follicle cell derivation arise in young patients and include pediatric type FL, testicular FL and a large B-cell lymphoma with IRF4 rearrangement. Historically DLBCL was separated into only two histological variants, centroblastic and immunoblastic. In 2017 the WHO classification recommended (1) the segregation of activated B cell and germinal center B cell derived DLBCL, (2) the identification of high-grade B-cell lymphoma with double hit, and (3) the recognition of an aggressive lymphoma that may resemble Burkitt lymphoma, currently designated in the International Consensus Classification as Large B-cell lymphoma with 11q aberration. Today we appreciate greater genomic complexity among aggressive B-cell lymphomas. Recent studies with NGS and mutational profiling have identified clinically significant genetic subgroups. It is hoped that these data ultimately will lead to targeted therapy based on the genetic profile.
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