First-Line Therapy, Autologous Stem-Cell Transplantation, and Post-Transplantation Maintenance in the Management of Newly Diagnosed Mantle Cell Lymphoma

Background: In Ontario, no clearly defined standard of care for the management of mantle cell lymphoma (mcl) has been developed, and substantial variability from centre to centre is evident. This guidance document was prompted by the need to harmonize practice in Ontario with respect to first-line, conditioning, and post-transplantation maintenance therapy for patients newly diagnosed with transplantation-eligible mcl. Methods: The medline and embase databases were systematically searched from January 2013 to January 2020 for evidence, and the best available evidence was used to draft recommendations relevant to first-line therapy, autologous stem-cell transplantation, and post-transplantation maintenance in the management of transplantation-eligible newly diagnosed mcl. Final approval of this guidance document was obtained from the Stem Cell Transplant Advisory Committee. Recommendations: These recommendations apply to all cases of transplantation-eligible newly diagnosed mcl: (1) Alternating cycles of r-chop (rituximab plus cyclophosphamide–doxorubicin–vincristine–prednisolone) and r-dhap [rituximab plus dexamethasone–high-dose cytarabine–cisplatin] is the recommended first-line treatment for symptomatic patients newly diagnosed with mcl before autologous stem-cell transplantation (asct). (2) Rituximab plus hyperfractionated cyclophosphamide–vincristine–doxorubicin–dexamethasone (r–hypercvad), alternating with methotrexate and cytarabine, is not recommended for the treatment of patients with newly diagnosed mcl. (3) beam (carmustine–etoposide–cytarabine–melphalan), beac (carmustine–etoposide–cytarabine–cyclophosphamide), and total-body irradiation–based regimens are reasonable conditioning options for patients with mcl who have responded to first-line therapy and who are undergoing asct. (4) Maintenance therapy with rituximab is recommended for patients with newly diagnosed mcl who have undergone asct.