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最后登录2025-5-6
  
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发表于 2023-9-13 10:35:14
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来自: 中国北京
In the 2017 WHO classification, these are included as a provisional entity designated as Burkitt-like lymphoma with 11q aberration. These subset of lymphomas have a clinical course similar to BL, but the optimum management of this rare subtype is undefined, though it is most often treated like typical BL. (在2017年WHO分类中,这些被列为具有11q畸变的伯基特样淋巴瘤的临时实体。这些淋巴瘤亚型的临床病程与伯基特淋巴瘤相似,但这种罕见亚型的最佳治疗方法尚不明确,尽管它通常像典型的伯基特淋巴瘤一样治疗。)
上面这段话摘自NCCN指南2023.2版,其中援引的是WHO分类第4版的2017年修订版。但是在WHO分类第5版中,这个类型被重新命名为高级别B细胞淋巴瘤伴11q异常,而且指出最近的研究显示这个类型的淋巴瘤在突变频谱上与伯基特淋巴瘤不一致,而更接近于生发中心亚型的弥漫大B细胞淋巴瘤,所以,标准方案是什么还有待更多的研究。
High-grade B-cell lymphoma with 11q aberration, formerly known as Burkitt-like lymphoma with 11q aberration in WHO-HAEM4R, is an aggressive MYC rearrangement-negative mature B-cell lymphoma with a morphology similar to Burkitt lymphoma (BL) or with an intermediate or blastoid appearance, an immunophenotype (CD10+, BCL6+, BCL2-), and/or gene expression profile (GEP) similar to BL, and a characteristic chromosome 11q-gain/loss pattern. More recent studies have also confirmed that the mutational spectrum, besides the pattern of genomic imbalances, is different from that of BL and is more similar to that of DLBCL of GCB type.
在与WHO唱对台戏的国际共识分类(ICC)中,对这个类型是这样描述的:
The 2016 WHO classification recognized the provisional entity, Burkitt-like lymphoma (BLL) with 11q aberration, identified originally as a lesion clinically and pathologically resembling Burkitt lymphoma (BL) but lacking MYC-R. The patients are more frequently children and young adults with a good prognosis. Subsequent studies have demonstrated the morphology and phenotype of these tumors to be more variable than originally described, including patients with mainly centroblastic-type large cells. Importantly, genetic studies also suggest the
disease is distinct from BL and is closer to conventional DLBCL with GCB derivation harboring more complex karyotypes and the absence of typical BL mutations. This provisional entity
has now been renamed “large B-cell lymphoma with 11q aberration” (2016年WHO分类确认了具有11q畸变的伯基特样淋巴瘤(BLL)这一临时实体,最初被确定为临床和病理上类似伯基特淋巴瘤(BL)但缺乏MYC重排的病变。患者多为儿童和年轻人,预后良好。随后的研究表明,这些肿瘤的形态和表型比最初描述的更具可变性,包括主要为中心母细胞样大细胞的患者。重要的是,遗传学研究还表明,该疾病与伯基特淋巴瘤不同,更接近传统的弥漫大B细胞淋巴瘤,来源于生发中心B细胞并具有更复杂的核型,并且没有典型的伯基特淋巴瘤相关突变。这个临时实体现在被重命名为“11q畸变的大B细胞淋巴瘤”)
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