Lymphomas with concurrent BCL2 and MYC translocations: the critical factors associated with survival
Lymphomas with concurrent BCL2 and MYC translocations: the critical factors associated with survival
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DOI：
10.1182/blood-2009-03-212191
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发表时间：
2009-09-10
期刊：
影响因子：
20.3
通讯作者：
Horsman, Douglas E.
中科院分区：
文献类型：
--
作者：
Johnson, Nathalie A.;Savage, Kerry J.;Horsman, Douglas E.
BCL2和MYC是淋巴瘤中常见的癌基因。通过核型和/或荧光原位杂交，在54个样本中发现了BCL2和MYC同时易位（BCL2(+)/MYC+），目的是将临床和细胞遗传学特征与总生存期联系起来。BCL2(+)/MYC+淋巴瘤诊断为无法分类的b细胞淋巴瘤（BCLU, n = 36），特征介于Burkitt淋巴瘤和弥漫大b细胞淋巴瘤（DLBCL）之间；DLBCL （n = 17）或滤泡性淋巴瘤（n = 1）。尽管存在t(14; 18)， 5例BCL2蛋白阴性。非免疫球蛋白基因/MYC（非ig /MYC）易位发生在54例患者中的24例（44%），与DLBCL形态高度相关（P < 0.001）。在中位5.3年的随访中，6名患者仍处于缓解期，32名患者在MYC+重排后6个月内死亡，无论MYC+是否在诊断时发生（54例中的31例）或转化（54例中的23例；P = 0.53）。非ig /MYC易位伴侣，缺乏BCL2蛋白表达，接受利妥昔单抗化疗，与更有利的结果相关，但低国际预后指数评分和DLBCL形态是总生存的独立预测因素。对所有侵袭性淋巴瘤的BCL2和MYC状态进行全面的细胞遗传学分析，可以确定一组高危患者，这些患者可能受益于包括利妥昔单抗和/或BCL2靶向治疗在内的化疗方案。（中国医学杂志。2009;114:2273-2279）
BCL2 and MYC are oncogenes commonly deregulated in lymphomas. Concurrent BCL2 and MYC translocations (BCL2(+)/MYC+) were identified in 54 samples by karyotype and/or fluorescence in situ hybridization with the aim of correlating clinical and cytogenetic characteristics to overall survival. BCL2(+)/MYC+ lymphomas were diagnosed as B-cell lymphoma unclassifiable (BCLU; n = 36) with features intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma (DLBCL); DLBCL (n = 17), or follicular lymphoma (n = 1). Despite the presence of a t(14; 18), 5 cases were BCL2 protein-negative. Nonimmunoglobulin gene/MYC (non-IG/MYC) translocations occurred in 24 of 54 cases (44%) and were highly associated with DLBCL morphology (P < .001). Over a median follow-up of 5.3 years, 6 patients remained in remission and 32 died within 6 months of the MYC+ rearrangement, irrespective of whether MYC+ occurred at diagnosis (31 of 54) or transformation ( 23 of 54; P = .53). A non-IG/MYC translocation partner, absent BCL2 protein expression and treatment with rituximab-based chemotherapy, were associated with a more favorable outcome, but a low International Prognostic Index score and DLBCL morphology were independent predictors of overall survival. A comprehensive cytogenetic analysis of BCL2 and MYC status on all aggressive lymphomas may identify a group of high-risk patients who may benefit from chemotherapeutic regimens that include rituximab and/or BCL2-targeted therapy. (Blood. 2009; 114: 2273-2279)