The clinical presentation and prognosis of diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC rearrangement
The clinical presentation and prognosis of diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC rearrangement
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DOI：
10.3324/haematol.11305
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发表时间：
2007-10-01
期刊：
影响因子：
10.1
通讯作者：
Avet-Loiseau, Herve
中科院分区：
文献类型：
--
作者：
Le Gouill, Steven;Talmant, Pascaline;Avet-Loiseau, Herve
背景和目的弥漫性大B细胞淋巴瘤（DLBCL）是一种常见的淋巴瘤，根据其基因表达模式可分为三个亚组。本研究的目的是描述DLBCL的临床，生物学，免疫表型和细胞遗传学特征，同时t（14;18）和8 q24/c-MYC rearrangement.Design和MethodsSixteen例DLBCL的双易位，确定在1998年和2006年1月之间。这些病例的临床特点进行了检查和形态学，免疫组化，流式细胞仪和细胞遗传学分析performed.ResultsAll患者有侵略性的功能：B症状（81%），ECOG体力状态>2（81%），乳酸脱氢酶升高（100%），IV期疾病（100%），至少有一个结外定位（骨髓、血液和中枢神经系统受累分别为93%、50%和50%），年龄校正的IPI评分为3分的患者占81%。尽管接受了强化化疗方案（包括同种异体移植），但所有患者均死于疾病进展。无进展生存期和总生存期分别为4个月和5个月。进行免疫表型分析（CD 20、CD 10、Bcl-6、Mum-1、Bcl-2、CD 138、MIB 1、CD 19、CD 5、CD 38和slg），显示DLBCL具有生发中心（GC）特征。Ki-67染色范围为70 - 90%。通过细胞遗传学分析[常规细胞遗传学和/或荧光原位杂交（FISH）]评估的所有病例均具有复杂的核型。在一例病例中，我们发现了一个以前从未在DLBCL中报道过的8 q24/c-MYC易位变异：t（8;9）（q24;p13）和t（14;18）（q32;q21）。BCL-6重排的FISH研究，发现重新安排在4 cases.Interpretation和ConclusionsIn结论，DLBCL并发t（14;18）和8 q24/c-MYC重排是一个亚组的GC-DLBCL与不良的结果。Bcl-2阳性的DLBCL伴异常侵袭性表现时是否存在双易位值得探讨。
Background and ObjectivesDiffuse large B-cell lymphomas (DLBCL) are common lymphomas that have been classified into three subgroups on the basis of their patterns of gene expression. The aim of this study was to characterize the clinical, biological, immunophenotypic and cytogenetic features of DLBCL with concurrent t(14;18) and 8q24/c-MYC rearrangement.Design and MethodsSixteen cases of DLBCL with the dual translocation were identified between 1998 and January 2006. The clinical features of these cases were examined and morphological, immunohistochemical, flow cytometric and cytogenetic analyses were performed.ResultsAll patients had aggressive features: B symptoms (81%), ECOG performance status >2 (81%), elevated lactate dehydrogenase (100%), stage IV disease (100%) with at least one extra-nodal localization (bone marrow, blood and central nervous system involvement in 93%, 50% and 50%, respectively) and age-adjusted IPI score of 3 in 81%. Despite intensive chemotherapy regimens (including allogeneic transplants), all patients died of disease progression. Progression-free and overall survival was 4 and 5 months, respectively. Immunophenotyping analysis (CD20, CD10, Bcl-6, Mum-1, Bcl-2 CD138, MIB1, CD19, CD5, CD38 and slg) was performed and showed DLBCL with a germinal center (GC) profile. Ki-67 staining ranged from 70 to 90%. All cases assessed by cytogenetics analysis [conventional cytogenetic and/or fluorescence in situ hybridization (FISH)] had a complex karyotype. In one case, we identified a 8q24/c-MYC translocation variant never reported in DLBCL before: t(8;9)(q24;p13) and t(14;18)(q32;q21). The BCL-6 rearrangement was investigated by FISH and found to rearranged in four cases.Interpretation and ConclusionsIn conclusion, DLBCL with concurrent t(14;18) and 8q24/c-MYC rearrangement is a subgroup of GC-DLBCLwith poor outcome. It is worth searching for the coexistence of dual translocations in Bcl-2-positive DLBCL with unusual aggressive presentation.