Abstract

Mantle cell lymphoma (MCL) is an aggressive B-cell lymphoma characterized by CD5+, CCND1+, and the CCND1-IGH translocation. Although patients with MCL respond, at least temporarily, to conventional chemotherapy, they eventually have a relapse and the prognosis is generally poor. As a primary treatment option for patients with untreated MCL, a rituximab-containing chemotherapy regimen is administered according to the patient's eligibility for high-dose chemotherapy followed by autologous stem cell transplantation (ASCT). For ASCT-eligible patients, an induction chemotherapy that includes high-dose cytarabine followed by ASCT is recommended. For ASCT-ineligible patients, a less intensive therapy, such as R-CHOP, bortezomib-containing therapy (VR-CAP), or bendamustine plus rituximab, is recommended. These induction therapies can be followed by rituximab maintenance. For those with relapsed/refractory MCL, fludarabine, bendamustine, 90Y-ibritumomab tiuxetan, and conventional cytotoxic agents can be used. Recently, ibrutinib, an inhibitor of Bruton tyrosine kinase, has been approved for relapsed/refractory MCL. Recent advances in the management of MCL will improve the prognosis of patients with MCL.

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