Mantle Cell Lymphoma: Treatment

Mantle Cell Lymphoma: overview

This algorithm divides mantle cell lymphoma treatment by fitness and line of therapy. For young or fit patients, the intensive approach is cytarabine-containing induction followed by autologous stem cell transplant and rituximab maintenance (LYMA). The chart also shows adding ibrutinib to induction (TRIANGLE); in the site's summary, ibrutinib-containing induction plus maintenance was superior to transplant alone, and transplant added no clear benefit when ibrutinib was given.

For older or less fit patients, the standard is bendamustine plus rituximab followed by rituximab maintenance (StiL), with BR plus ibrutinib as the BTK inhibitor-added option (SHINE). SHINE prolonged PFS by about 28 months but without an OS benefit and with more atrial fibrillation, bleeding and infection. For relapsed or refractory disease, the chart lists a covalent BTK inhibitor or pirtobrutinib (BRUIN), and the CD19 CAR-T product brexucabtagene autoleucel (ZUMA-2), which produced a 93% response rate and 67% complete response rate after prior BTK inhibitor therapy, with higher ICANS rates than in DLBCL.

Trials behind this algorithm

• TRIANGLE Dreyling M et al, Lancet, 2024; PMID: 38705160
• StiL trial Rummel MJ et al, 2013, Lancet, PMI:D 23433739
• SHINE Wang ML et al, NEJM, 2022; PMID: 35657079
• ZUMA-2 Wang M et al, NEJM, 2020; PMID: 32242358

Background reading: Mantle cell lymphoma (MCL) board review notes.