Yazarlar : Moreno SM, Montalbán C, Piris MA.

Yayın : Leuk Lymphoma.

Yayın Yılı : 2011

Pubmed Linki : Large B Cell Lymphomas with Plasmablastic Differentiation: A Biological and Therapeutic Challenge.

Konu : Lenfoma

Literatür İçeriği :  

Abstract

Abstract Plasmablastic differentiation can be found in a variety of Large B Cell Lymphomas, including Plasmablastic Lymphoma, ALK-Positive Large B Cell Lymphoma, Primary Effusion Lymphoma, Large B Cell Lymphoma arising in HHV-8-associated Multicentric Castleman Disease and Diffuse Large B Cell Lymphoma with partial plasmablastic phenotype. These tumors are characterized by the acquisition of the transcriptional profile of plasma cells (with overexpression of PRDM1/Blimp1 and XBP1s, in concert with the extinction of the B cell differentiation program) by proliferating immunoblasts. This particular biological entity, i.e., Large B Cell Lymphoma with Plasmablastic Differentiation, is almost always associated with an aggressive clinical behavior. This review summarizes the current knowledge of the biological basis of plasmablastic differentiation in Large B Cell Lymphomas, the diagnostic borders with DLBCL and Multiple Myeloma, the associated adverse molecular events (with concomitant MYC, p53 and ALK alterations), and the potential therapeutic targets so far identified (including the Unfolded Protein Response Pathway). The highly aggressive nature of these lymphomas and the relative paucity of molecular data available, highlights the need for deeper insights into the molecular pathogenesis of Large B Cell Lymphomas with Plasmablastic Differentiation in order to identify new and effective alternative treatments.

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