Aggressive Leukemic Non-Nodal Mantle Cell Lymphoma With P53 Gene Rearrangement/Mutation is Highly Responsive to Rituximab/Ibrutinib Combination Therapy

. Non-nodal leukemicmantle cell lymphoma (NN-L-MCL) is a relatively uncommon subtype of mantle cell lymphoma (MCL), which is generally considered to be indolent in nature and its recognition is important to avoid overtreatment. . A subset of these patients present with rapidly progressing and aggressive disease but there are currently no standard guidelines in treatment of these patients. . We describe 2 patients with aggressive NN-L-MCL who had presented with symptomatic disease. Cytogenetic and molecular evaluations suggested disruptions in the TP53 gene. . Both patients were successfully treated with rituxan with ibrutinib followed by autologous stem cell transplantation and currently remain disease-free at 18 months post-transplantation. . Although P53 alterations are associated with poor outcome in MCL overall, the response to chemotherapy-free treatment with rituximab with ibrutinib in NN-L-MCL with TP53 disruption is encouraging. (C) 2018 Elsevier Inc. All rights reserved.