Plasmablastic lymphoma: from genetics to treatment

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.

Keywords:

• Plasmablastic lymphoma
• JAK-STAT
• RAS-RAF
• NOTCH
• IRF4
• MYC

Author contributions

Fabian Frontzek, Stephan Hailfinger, and Georg Lenz conceptualized and wrote this review article.

Disclosure statement

Fabian Frontzek and Stephan Hailfinger have no potential conflicts of interest. Georg Lenz received research grants not related to this manuscript from AGIOS, AQUINOX, AstraZeneca, Bayer, Celgene, Gilead, Janssen, Morphosys, Novartis, Roche, and Verastem. Georg Lenz received honoraria not related to this manuscript from ADC Therapeutics, Abbvie, Amgen, AstraZeneca, Bayer, BMS, Celgene, Constellation, Genmab, Gilead, Incyte, Janssen, Karyopharm, Miltenyi, Morphosys, NanoString, Novartis, and Roche.