Sanders Lucinda, Jayne Sandrine, Kennedy Ben, Miall Fiona, Aukema Sietse M, Siebert Reiner, Wagner Simon D, Dyer Martin J S
Department of Haematology, University Hospitals of Leicester, Leicester LE1 5WW, UK.
Ernest and Helen Scott Haematological Research Institute and Department of Biochemistry, University of Leicester, Leicester LE1 9HN, UK.
Case Rep Hematol. 2014;2014:120714. doi: 10.1155/2014/120714. Epub 2014 Oct 1.
The WHO classification of lymphomas allows for a group of diseases that have features intermediate between those of Burkitt lymphoma and diffuse large B-cell lymphoma. These are a diverse group of diseases whose genetics and clinical course are yet to be fully described. We report an unusual case of high grade B-cell lymphoma, intermediate between DLBCL and BL, lacking CD10 expression in which the chromosomal translocation t(3;8)(q27;q24) was found to be the sole chromosomal abnormality. FISH analysis demonstrated juxtaposition of the BCL6 and MYC loci without obvious involvement of the IGH locus, suggesting constitutive MYC expression due to promoter substitution. The patient responded to intensive chemotherapy and remains in remission two years after finishing therapy.
世界卫生组织（WHO）对淋巴瘤的分类中包括一组疾病，其特征介于伯基特淋巴瘤和弥漫性大B细胞淋巴瘤之间。这是一组多样的疾病，其遗传学和临床病程尚未完全阐明。我们报告了一例罕见的高级别B细胞淋巴瘤病例，介于弥漫性大B细胞淋巴瘤和伯基特淋巴瘤之间，缺乏CD10表达，其中发现染色体易位t(3;8)(q27;q24)是唯一的染色体异常。荧光原位杂交（FISH）分析显示BCL6和MYC基因座并列，而IGH基因座未明显受累，提示由于启动子替代导致MYC组成性表达。该患者对强化化疗有反应，完成治疗两年后仍处于缓解状态。
