Plasmablastic lymphoma: from genetics to treatment. Issue 4 (21st March 2023)

• Record Type: Journal Article
• Authors: Frontzek, Fabian; Hailfinger, Stephan; Lenz, Georg
• Is Part Of: Leukemia & lymphoma. Volume 64:Issue 4(2023)
• Page Start: 799
• Page End: 807
• Publication Date: 2023-03-21
• Subjects: Plasmablastic lymphoma -- JAK-STAT -- RAS-RAF -- NOTCH -- IRF4 -- MYC; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2022.2162341
• Languages: English
• ISSNs: 1042-8194

Abstract

Abstract: Plasmablastic lymphoma (PBL) represents a rare distinct lymphoma entity with plasmablastic morphology and plasmacytic immunophenotype that is characterized by an aggressive clinical course. Standard chemotherapeutic regimens often remain insufficient to cure affected patients. Recently, comprehensive molecular analyses of large cohorts of primary PBL samples have revealed the mutational landscape as well as the pattern of copy number alterations of this rare lymphoma subtype. Identification of recurrent aberrations affecting the JAK-STAT, RAS-RAF, NOTCH, IRF4, and MYC signaling pathways drive the molecular pathogenesis of PBL and hold great potential for novel targeted therapeutic approaches.