High incidence of MYC and BCL2 abnormalities in mantle cell lymphoma, although only MYC abnormality predicts poor survival.
High incidence of MYC and BCL2 abnormalities in mantle cell lymphoma, although only MYC abnormality predicts poor survival.
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套细胞淋巴瘤中 MYC 和 BCL2 异常的发生率很高，但仅 MYC 异常预示生存率较差
DOI：
10.18632/oncotarget.5705
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发表时间：
2015-12-08
期刊：
影响因子：
--
通讯作者：
Qiu L
中科院分区：
文献类型：
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作者：
Yi S;Zou D;Li C;Zhong S;Chen W;Li Z;Xiong W;Liu W;Liu E;Cui R;Ru K;Zhang P;Xu Y;An G;Lv R;Qi J;Wang J;Cheng T;Qiu L
成熟B细胞淋巴瘤中MYC和BCL 2重排的发生率和预后作用已被广泛研究，除了罕见的套细胞淋巴瘤（MCL）。在这里，我们分析了MYC和BCL 2异常和其他细胞遗传学畸变的荧光原位杂交（FISH）在50例MCL患者骨髓受累。18例患者（36.0%）有MYC增益和/或扩增，12例患者（24.0%）有BCL 2增益和/或扩增。在18例MYC异常患者中，4例同时发生MYC易位，但在BCL 2异常患者中未检测到BCL 2易位。只有2例患者（4.0%）同时存在MYC和BCL 2异常。与无此异常的患者相比，MYC异常的患者具有显著更高的肿瘤负荷、更高的中/高风险MIPI组百分比和基因组不稳定性。然而，在临床和细胞遗传学因素方面，没有观察到有或没有BCL 2异常的患者之间的显着差异。MYC异常患者的无进展生存期（PFS）较差（9.0 vs. 48.0个月，p = .000）和总生存期（OS）（12.0 vs.94.5个月，p = .000），但BCL 2异常的存在并不显著影响PFS或OS。在多变量分析中，MYC异常是影响PFS和OS的独立不良因素，强化化疗并不能改善这些患者的预后。因此，MYC而不是BCL 2异常的存在预测MCL患者的生存率差，应该为这些患者开发新的治疗策略。
The incidence and prognostic role of MYC and BCL2 rearrangements in mature B-cell lymphomas have been extensively studied, except the infrequent mantle cell lymphoma (MCL). Here, we analyzed the MYC and BCL2 abnormalities and other cytogenetic aberrations by fluorescence in situ hybridization (FISH) in 50 MCL patients with bone marrow involvement. Eighteen patients (36.0%) had MYC gains and/or amplifications, and twelve patients (24.0%) had BCL2 gains and/or amplifications. Among the 18 patients with MYC abnormality, four had simultaneous MYC translocations, but no BCL2 translocation was detected among patients with BCL2 abnormality. Only two patients (4.0%) had both MYC and BCL2 abnormalities. The patients with a MYC abnormality had a significantly higher tumor burden, a higher percentage of medium/high risk MIPI group and genomic instability compared to those without this abnormality. However, no significant difference was observed between patients with or without a BCL2 abnormality in terms of clinical and cytogenetic factors. Patients with a MYC abnormality had poorer progress-free survival (PFS) (9.0 vs. 48.0 months, p = .000) and overall survival (OS) (12.0 vs. 94.5 months, p = .000), but the presence of a BCL2 abnormality did not significantly influence either PFS or OS. In multivariate analysis, the MYC abnormality was the independent adverse factor for both PFS and OS, and intensive chemotherapy did not improve the outcome of these patients. Thus, the presence of a MYC but not BCL2 abnormality predicted the poor survival of MCL patients, and a new treatment strategy should be developed for these patients.