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BCL3基因在两名慢性淋巴细胞白血病患者中的作用。

Involvement of the BCL3 gene in two patients with chronic lymphocytic leukemia.

作者信息

Yabumoto K, Ohno H, Doi S, Edamura S, Arita Y, Akasaka T, Matsumoto J, Kadowaki N, Fukuhara S, Okuma M

机构信息

First Department of Internal Medicine, Faculty of Medicine, Kyoto University, Japan.

出版信息

Int J Hematol. 1994 Apr;59(3):211-8.

PMID:8011990
Abstract

The t(14;19)(q32;q13) is a recurring translocation found in some patients with chronic lymphocytic leukemia (CLL), and the t(14;19) juxtaposes the BCL3 gene on chromosome 19 with the immunoglobulin heavy chain gene (IGH) locus on chromosome 14. Genomic DNAs from 49 patients with chronic B-cell leukemia and the related lymphomas were examined by Southern blot hybridization using 2 separate probes, named p alpha 1.4P and p alpha .5B, from the BCL3 gene locus. None of the 18 patients with leukemic manifestations of non-Hodgkin's lymphomas had detectable BCL3 rearrangements. Of 31 patients with CLL, 2 had the BCL3 rearrangements. A comigration study using the C alpha and C epsilon constant gene probe from IGH indicated that the t(14;19) translocation occurred in these 2 patients, and they were diagnosed with CLL/prolymphocytic (PL) according to the French-American-British (FAB) classification. Probes for the IGH locus revealed that leukemia cells of the 2 patients each were clonal, indicating that both small lymphocytes and prolymphocytoid cells found in the peripheral blood of one patient had the t(14;19), as well as a major population of the small lymphocytes in the peripheral blood of a second patient. It thus appears that tumor cells carrying the t(14;19) constitute a distinct disease entity in a group of chronic B-cell leukemia, that has a converting potential to more aggressive forms.

摘要

t(14;19)(q32;q13)是在一些慢性淋巴细胞白血病(CLL)患者中发现的一种反复出现的易位,t(14;19)使19号染色体上的BCL3基因与14号染色体上的免疫球蛋白重链基因(IGH)位点并列。使用来自BCL3基因位点的2个单独探针p alpha 1.4P和p alpha.5B,通过Southern印迹杂交对49例慢性B细胞白血病及相关淋巴瘤患者的基因组DNA进行检测。18例非霍奇金淋巴瘤白血病表现患者中均未检测到BCL3重排。在31例CLL患者中,2例有BCL3重排。使用来自IGH的C alpha和C epsilon恒定基因探针进行的共迁移研究表明,这2例患者发生了t(14;19)易位,根据法国-美国-英国(FAB)分类,他们被诊断为CLL/幼淋巴细胞白血病(PL)。IGH位点的探针显示,这2例患者的白血病细胞均为克隆性,表明1例患者外周血中发现的小淋巴细胞和幼淋巴细胞样细胞均有t(14;19),另一例患者外周血中的主要小淋巴细胞群也有t(14;19)。因此,携带t(14;19)的肿瘤细胞似乎在一组慢性B细胞白血病中构成了一个独特的疾病实体,具有向更具侵袭性形式转化的潜力。

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Involvement of the BCL3 gene in two patients with chronic lymphocytic leukemia.BCL3基因在两名慢性淋巴细胞白血病患者中的作用。
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Reappraisal of BCL3 as a molecular marker of anaplastic large cell lymphoma.
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p53 mutation in B-cell lymphoid neoplasms with reference to oncogene rearrangements associated with chromosomal translocations.B细胞淋巴瘤中的p53突变与染色体易位相关的癌基因重排的关系
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Mol Cell Biol. 1996 Apr;16(4):1342-8. doi: 10.1128/MCB.16.4.1342.