Michaux L, Dierlamm J, Wlodarska I, Bours V, Van den Berghe H, Hagemeijer A
Department of Hematology, Cliniques Universitaires UCL Saint Luc, Bruxelles, Belgium.
Cancer Genet Cytogenet. 1997 Mar;94(1):36-43. doi: 10.1016/s0165-4608(96)00247-6.
The t(14;19)(q32.3;q13.2) is a rare but recurrent translocation found in patients with B-cell malignancies, mainly in chronic B-cell lymphoproliferative disorders. When occurring in chronic lymphocytic leukemia (CLL), atypical lymphocyte morphology and immunophenotype have been reported. A high proportion of patients with CLL and t(14;19) are aged less than 40 years. t(14;19) is often associated with rapidly progressive disease, and overall prognosis is poor compared to the expected survival in chronic lymphocytic leukemia and low-grade B-cell lymphoma. t(14;19) is rarely the sole cytogenetic aberration. Trisomy 12 is the most frequent associated abnormality, and is observed in 50% of cases. t(14;19) involves the BCL3 gene, which is located at the breakpoint on chromosome 19 and is juxtaposed to the immunoglobulin heavy chain gene locus on chromosome 14 (often in the switch alpha region) in a "head-to-head" configuration. The translocation does not interrupt the transcriptional integrity of BCL3, but is associated with overexpression of this gene, which encodes an I kappa B-like protein and modulates the activity of the NF-kappa B transcription factors. The genes affected by overexpression of BCL3 remain to be identified.
t(14;19)(q32.3;q13.2)是一种罕见但反复出现的易位，见于B细胞恶性肿瘤患者，主要是慢性B细胞淋巴细胞增殖性疾病。当发生在慢性淋巴细胞白血病（CLL）时，已有非典型淋巴细胞形态和免疫表型的报道。高比例的CLL和t(14;19)患者年龄小于40岁。t(14;19)常与疾病快速进展相关，与慢性淋巴细胞白血病和低级别B细胞淋巴瘤的预期生存期相比，总体预后较差。t(14;19)很少是唯一的细胞遗传学异常。三体12是最常见的相关异常，见于50%的病例。t(14;19)涉及BCL3基因，该基因位于19号染色体的断点处，并以“头对头”构型与14号染色体上的免疫球蛋白重链基因座（常位于转换α区域）并列。该易位不中断BCL3的转录完整性，但与该基因的过表达相关，该基因编码一种IκB样蛋白并调节NF-κB转录因子的活性。受BCL3过表达影响的基因仍有待确定。
