Sevilla Deborah W, Nandula Subhadra V, Colovai Adriana I, Alexander Suzy, Murty Vundavalli V, Alobeid Bachir, Bhagat Govind
Department of Pathology, Columbia University, New York, NY 10032, USA.
Hum Pathol. 2009 Apr;40(4):588-93. doi: 10.1016/j.humpath.2008.08.012. Epub 2008 Nov 7.
Cytogenetic abnormalities of chromosome 12p involving the TEL/ETV6 gene are observed in a variety of hematopoietic neoplasms including acute leukemias, myelodysplastic syndromes, and myeloproliferative disorders. Karyotypic aberrations, including rearrangements, deletions, and amplifications of chromosome 12p, have been documented in B-cell non-Hodgkin lymphoma; however, rearrangements targeting TEL have rarely been reported. Here we describe a diffuse large B-cell lymphoma that had a complex karyotype including t(9;12)(q22;p13), which was confirmed by fluorescence in situ hybridization to represent rearrangement of TEL. Additional cytogenetic abnormalities included t(3;14)(q27;q32) involving the variant, alternative breakpoint region of the BCL6 gene and del(6)(q13q23), resulting in the loss of 1 allele of BLIMP1. This case reiterates the importance of correlating morphologic and phenotypic findings with the results of cytogenetic analysis to avoid errors in diagnosing hematologic neoplasms and highlights the rare association of B-cell non-Hodgkin lymphoma with aberrations of TEL.
在包括急性白血病、骨髓增生异常综合征和骨髓增殖性疾病在内的多种造血系统肿瘤中,均观察到涉及TEL/ETV6基因的12号染色体短臂的细胞遗传学异常。在B细胞非霍奇金淋巴瘤中已记录到包括12号染色体短臂重排、缺失和扩增在内的核型畸变;然而,针对TEL的重排很少被报道。在此,我们描述了1例弥漫性大B细胞淋巴瘤,其具有复杂核型,包括t(9;12)(q22;p13),荧光原位杂交证实该核型代表TEL重排。其他细胞遗传学异常包括涉及BCL6基因变异、替代断裂点区域的t(3;14)(q27;q32)和del(6)(q13q23),导致BLIMP1的1个等位基因缺失。该病例重申了将形态学和表型结果与细胞遗传学分析结果相关联以避免血液系统肿瘤诊断错误的重要性,并突出了B细胞非霍奇金淋巴瘤与TEL畸变的罕见关联。