Boudjarane John, Essaydi Arnaud, Farnault Laure, Popovici Cornel, Lafage-Pochitaloff Marina, Beaufils Nathalie, Berda-Haddad Yaël, Lacroix Romaric, Nicolino-Brunet Corinne, Le Treut Thérèse, Zattara Hélène, Gabert Jean, Kahn-Perlès Brigitte, Costello Régis
Département de Génétique Médicale, Hôpital La Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France; Aix-Marseille Université, Marseille, France.
Exp Dermatol. 2015 Jan;24(1):60-2. doi: 10.1111/exd.12567. Epub 2014 Nov 13.
Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of lymphomas primarily involving the skin. The most common types are mycosis fungoides (MF) and Sezary Syndrome (SS). We report a novel long-term fast-growing SS line termed BKP1 that was characterized by flow cytometry (FC), conventional and molecular cytogenetic [FISH/multi-FISH together with array comparative genomic hybridization (aCGH)]. FC immunophenotype of the BKP1 is CD2+CD5+CD3+CD4+CD8-CD7-CD25-CD26-CD30-CD158k+. The TCRγ characterization of BKP1 by PCR identified a clonal rearrangement. The conventional cytogenetic and Multi-FISH analysis showed complex chromosomal rearrangements. aCGH analysis highlighted the loss of genes involved in cell cycle control, in immune response (HLA, complement complex) and DNA damage repair mechanisms. The BKP1 is another lymphoma cell line thoroughly characterized that can be a valuable tool for both basic and applied research such as identification of deregulated genes and/or pathways and screening for new antilymphoma drugs.
皮肤T细胞淋巴瘤(CTCL)是一组主要累及皮肤的异质性淋巴瘤。最常见的类型是蕈样肉芽肿(MF)和塞扎里综合征(SS)。我们报告了一种新的长期快速生长的SS细胞系,称为BKP1,通过流式细胞术(FC)、传统和分子细胞遗传学[荧光原位杂交/多重荧光原位杂交以及阵列比较基因组杂交(aCGH)]对其进行了表征。BKP1的FC免疫表型为CD2 + CD5 + CD3 + CD4 + CD8 - CD7 - CD25 - CD26 - CD30 - CD158k +。通过PCR对BKP1的TCRγ进行表征,确定了克隆重排。传统细胞遗传学和多重荧光原位杂交分析显示存在复杂的染色体重排。aCGH分析突出了参与细胞周期调控、免疫反应(HLA、补体复合物)和DNA损伤修复机制的基因缺失。BKP1是另一种经过全面表征的淋巴瘤细胞系,对于基础研究和应用研究都是一种有价值的工具,例如用于鉴定失调的基因和/或信号通路以及筛选新的抗淋巴瘤药物。