Summary of Therapy for MCL

The following agents, alone or in combination, represent targeted biological therapy options that may enable chemotherapy-free treatment strategies for most patients with MCL:[3]

Chemoimmunotherapy with BR (bendamustine and rituximab) or with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone)/R-DHAP (rituximab, dexamethasone, high-dose cytarabine, and cisplatin) is still considered a standard option for younger fit patients. Most patients can avoid autologous stem cell transplant (SCT), but maintenance therapy with rituximab for at least 2 to 3 years remains the standard of care after first-line induction therapy. The highest-risk patients may best respond to combinations of biological targeted therapies, followed by consolidation with allogeneic SCT or chimeric antigen receptor T-cell therapy.[4,5]

Routine administration of central nervous system (CNS) prophylaxis in patients with high-risk MCL has never been studied in a prospective randomized trial. The use of intrathecal or intravenous high-dose methotrexate or the use of systemic therapies with CNS penetration such as BTK inhibitors, high-dose cytarabine, or venetoclax have not been studied or proven efficacious in this situation.[6]

Outside the context of clinical trials, the use of measurable residual disease (MRD) testing has not been shown to be predictive for directing therapy for patients with MCL. In a retrospective analysis of a prospective randomized clinical trial, while MRD negativity after rituximab maintenance therapy was prognostic for a better outcome, continuation of maintenance rituximab prolonged progression-free survival and overall survival the most among patients with MRD-negative disease.[7][Level of evidence C1] Stopping maintenance rituximab was not indicated in patients with MRD-negative disease, negating any possible change in therapy based on that status.