Upfront Management of Blastoid Variant Mantle Cell Lymphoma: Making the Case for 2nd/3rd‐Generation Bruton Tyrosine Kinase Inhibitor‐Based Therapies

Abstract

ABSTRACT Introduction Blastoid variant‐mantle cell lymphoma (BV‐MCL) represents an aggressive subset of patients with no established standard of care treatment approach. Methods We performed a retrospective analysis of the clinical characteristics and outcomes of 17 de novo BV‐MCL patients treated at our institution between May 2009 and September 2023. In addition, we reviewed the literature supporting 2nd/3rd generation Bruton's Tyrosine‐kinase (BTKi)‐based therapies for upfront management. Results The complete response rate to frontline and salvage therapy was 66.7% and 25%, respectively. Two‐year overall survival (OS) was low at 62.5% (95% CI, 34.7%–81.1%). Variables associated with higher OS included receipt of consolidative HSCT (p = 0.017), TP53‐wild type (p = 0.031), intermediate‐ versus high‐risk Mantle Cell Lymphoma Prognostic Index score (p = 0.026), and achieving complete response with induction therapy (p = 0.027). Discussion Treatment outcomes with chemo‐immunotherapy in BV‐MCL patients are poor, especially among TP53‐mutated patients. Recent findings describing positive outcomes with novel BTKi‐based therapies are encouraging and should be considered in this high‐risk population.

Keywords

• blastoid variant
• Bruton tyrosine kinase inhibitor
• mantle cell lymphoma