Gebauer Niklas, Bernard Veronica, Gebauer Wolfgang, Thorns Christoph, Feller Alfred C, Merz Hartmut
Department of Pathology, Reference Center for Lymph Node Pathology and Hematopathology, University Hospital of Schleswig-Holstein , Luebeck , Germany.
Leuk Lymphoma. 2015 Jan;56(1):179-85. doi: 10.3109/10428194.2014.907896. Epub 2014 May 7.
Double-hit lymphomas (DHL) with MYC and either BCL2 or BCL6 rearrangements are rare neoplasms with an aggressive clinical presentation and grim prognosis. Moreover, molecular characterization of DHL remains insufficient, and especially the role of TP53 pathway disruption is unknown. We employed a next-generation sequencing approach to investigate the mutational status of TP53 in DHL and correlated genomic data with immunohistochemical reactivity for p53. We identified TP53 mutations in MYC+/BCL2+ lymphomas at a frequency intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma. Remarkably, TP53 mutations were particularly scarce in MYC+/BCL6+ lymphomas. Our findings indicate a significant difference between these two types of DHL at a molecular level with pathogenetic implications, as arguably, TP53 mutations inhibiting p53 mediated promotion of apoptosis pose a synergistic advantage in clonal evolution of cells with malignantly enforced overexpression of BCL2. Immunohistochemical staining appears to be a sensitive surrogate of TP53 mutation status with moderate specificity.
伴有MYC重排以及BCL2或BCL6重排的双打击淋巴瘤(DHL)是罕见肿瘤,临床表现侵袭性强,预后不佳。此外,DHL的分子特征仍不充分,尤其是TP53通路破坏的作用尚不清楚。我们采用新一代测序方法研究DHL中TP53的突变状态,并将基因组数据与p53的免疫组化反应性相关联。我们在MYC+/BCL2+淋巴瘤中鉴定出TP53突变,其频率介于弥漫性大B细胞淋巴瘤(DLBCL)和伯基特淋巴瘤之间。值得注意的是,TP53突变在MYC+/BCL6+淋巴瘤中尤为罕见。我们的研究结果表明,这两种类型的DHL在分子水平上存在显著差异,具有致病意义,因为可以说,抑制p53介导的细胞凋亡促进作用的TP53突变在BCL2恶性强制过表达的细胞克隆进化中具有协同优势。免疫组化染色似乎是TP53突变状态的敏感替代指标,特异性中等。