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

Publication date: Available online 13 November 2018Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Shahram Mori, Rushang D. Patel, Sarfraz Ahmad, Juan Varela, Tori Smith, Rola Altoos, Qi Shen, Steven C. Goldstein, Daniel O. PerskyAbstractClassical Mantle cell lymphoma (cMCL) is a B-cell neoplasm characterized immunohistochemically by their CD5+, CD19+, CD20+, CD23- and cyclinD1+ nature and confirmed genetically by the presence of t(11;14), resulting in the over-expression of Cyclin D1. Classical MCL generally presents with adenopathy, spleen, bone marrow involvement and extra-nodal sites most commonly the GI tract. Leukemic, Non-Nodal Mantle Cell Lymphoma (L-NN-MCL) is a relatively uncommon subtype of MCL, which is generally considered to be indolent in nature. Currently, there are no guidelines for risk-stratifying L-NN-MCL patients. Herein, we report two cases of L-NN-MCL who presented with the progressive disease requiring treatment. In case 1, the patient presented with complex cytogenetics including re-arrangements of chromosome 17, which were suggestive of the loss of P53 gene. Cytogenetic and DNA sequencing studies in case 2 revealed missense mutations in TP53 and KMT2A (MLL) genes, as well as a frameshift mutation in the BCL6 interacting co-repressor (BCOR) gene. Both patients received rituximab 375 mg/m2 monthly with ibrutinib 560 mg daily with normalization of blood counts within 2-months and achieved a complete remission prior to autologous stem cell trans...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research